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 Adekunle M. Adesina
Tarik Tihan
Christine E. Fuller
Tina Young Poussaint
Editors

Atlas of
Pediatric Brain Tumors

Second Edition

123
Atlas of Pediatric Brain Tumors
Adekunle M. Adesina • Tarik Tihan
Christine E. Fuller • Tina Young Poussaint
Editors

Atlas of Pediatric Brain

Tumors
Second Edition
Editors
Adekunle M. Adesina Tarik Tihan
Department of Pathology Department of Pathology
Texas Children’s Hospital Neuropathology Division
Baylor College of Medicine San Francisco, CA, USA
Houston, TX, USA
Tina Young Poussaint
Christine E. Fuller PBTC Neuroimaging Center
Division of Pathology and Laboratory Medicine Harvard Medical School
Cincinnati Children’s Hospital Medical Center Boston, MA, USA
Cincinnati, OH, USA

ISBN 978-3-319-33430-1 ISBN 978-3-319-33432-5 (eBook)

DOI 10.1007/978-3-319-33432-5

Library of Congress Control Number: 2016947963

© Springer International Publishing AG 2010, 2016

This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is
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Printed on acid-free paper

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The registered company is Springer International Publishing AG Switzerland
Preface to the First Edition

In the presentation of this atlas of pediatric brain tumors, we have followed the 2007 World
Health Organization (WHO) classification of brain tumors. We do recognize that there are
some tumor types that do not fit nicely into this classification. It is also critically important to
recognize that pediatric tumors within the same histological category are significantly different
from those occurring in adults. For example, a rare but increasingly recognized group of malig-
nant glioneuronal tumors with prominent epithelioid morphology is discussed separately in
Chap. 23 under the presumptive category of “malignant epithelioid glioneuronal tumors.” We
believe that this group of neoplasms has histologic characteristics distinctly different from
anaplastic gangliogliomas or primitive neuroectodermal tumors. We expect that as more cases
are reported, this group of malignant tumors may be recognized as a distinct entity or group of
entities in future WHO classification schemes.
In compiling the chapters, we have distilled only the important diagnostic information includ-
ing clinical, neuroimaging, and histologic features with an emphasis on the recognized variants
and their differential diagnoses. A section on molecular pathology and electron microscopy is
also included for each tumor category. A list of classic reviews and landmark articles on each of
the tumor entities is provided as suggested reading at the end of each chapter. The last chapter
represents a list of interesting and unusual cases for a diagnostic exercise by the reader.
In contrast to the more limited pathologic variations associated with adult brain tumors,
most pathologists are often aware of the extreme diversity and florid phenotypic variation
associated with pediatric brain tumors. The rarity of some of these phenotypic variants often
makes the definitive diagnosis of each of these entities a diagnostic challenge even for the
experienced pediatric pathologist. Those who have limited experience in pediatric neuropa-
thology are acutely aware of the fact that the ultimate definition of each specific tumor is not
without therapeutic and/or prognostic implications. This atlas is born out of the need to fill a
“void” in the practice of pediatric neuropathology. It is designed to provide a practical and
well-illustrated “beside-the-microscope” resource for the diagnostic pathologist who is called
upon to make critical decisions on sometimes tiny and often very “taxing” specimens.
As in every field of diagnostic pathology, a picture is worth a thousand words. We hope that
the bullet format used for its text will make for easy identification of relevant information that
will aid in the evaluation of clinical cases at the microscope. We have also included a signifi-
cant number of neuroradiological images that provide the critical correlations that are crucial
for recognizing some of the entities. We anticipate that the format and the illustrations will
allow easy use as a practical text for practitioners and trainees in neuropathology, neuroradiol-
ogy, neurooncology, and general surgical pathology.
This book is dedicated to all the children with brain tumors who are the inspiration for our
work. Their challenging tumors have shaped our practice experiences, and their courageous
struggle for survival has given us encouragement. To them, we remain forever indebted.

Houston, TX, USA Adekunle M. Adesina

Cincinnati, OH, USA Christine E. Fuller
San Francisco, CA, USA Tarik Tihan
Boston, MA, USA Tina Young Poussaint

v
Preface to the Second Edition

It has been six years since the publication of the first edition of the Atlas of Pediatric Brain
Tumors. In putting together this new edition, we have had the enriching experience of observ-
ing an explosion in the understanding of the differences in the genetic signatures of tumor
groups and subgroups, with implications for differing tumor biology, response to therapy, and
prognosis. In addition, these expression signatures show good correlation with specific signal-
ing pathway activation, represent prognostic markers, and serve as specific therapeutic targets.
These developments have been presented succinctly in the context of underlying morphology,
and we hope you find this presentation enriching.

Houston, TX, USA Adekunle M. Adesina

Cincinnati, OH, USA Christine E. Fuller
San Francisco, CA, USA Tarik Tihan
Boston, MA, USA Tina Young Poussaint

vii
Contents

1 Introduction and Overview of Brain Tumors ........................................................ 1

Adekunle M. Adesina

Part I Glial Tumors

2 Pilocytic Astrocytoma and Pilomyxoid Astrocytoma ............................................ 7
Christine E. Fuller
3 Pleomorphic Xanthoastrocytoma ........................................................................... 19
Christine E. Fuller
4 Infiltrative Astrocytomas (Diffuse Astrocytoma,
Anaplastic Astrocytoma, Glioblastoma) ................................................................ 25
Christine E. Fuller
5 Oligodendroglial Tumors......................................................................................... 43
Hope T. Richard and Christine E. Fuller
6 Ependymal Tumors .................................................................................................. 53
Christine E. Fuller

Part II Embryonal Neuroepithelial Tumors

7 Central Nervous System Primitive Neuroectodermal Tumors (PNETs)
and Medulloepithelioma .......................................................................................... 71
Adekunle M. Adesina, Jill V. Hunter, and Lucy Balian Rorke-Adams
8 Medulloblastoma ...................................................................................................... 81
Adekunle M. Adesina and Jill V. Hunter
9 Atypical Teratoid/Rhabdoid Tumor ....................................................................... 99
Adekunle M. Adesina, Jill V. Hunter, and Lucy Balian Rorke-Adams

Part III Tumors of the Meninges

10 Meningiomas ............................................................................................................ 113
Christine E. Fuller
11 Hemangiopericytoma and Other Mesenchymal Tumors ..................................... 129
Christine E. Fuller
12 Melanocytic Lesions ................................................................................................. 137
Christine E. Fuller
13 Hemangioblastoma .................................................................................................. 143
Hope T. Richard and Christine E. Fuller

ix
x Contents

Part IV Tumors of Cranial and Spinal Nerves

14 Schwannoma............................................................................................................. 151
Tarik Tihan
15 Neurofibroma ........................................................................................................... 157
Tarik Tihan
16 Malignant Peripheral Nerve Sheath Tumors ........................................................ 163
Yazgi Koy and Tarik Tihan

Part V Neuronal and Mixed Neuronal-Glial Tumors

17 Desmoplastic Infantile Astrocytoma and Ganglioglioma ..................................... 171
Adekunle M. Adesina
18 Dysembryoplastic Neuroepithelial Tumor ............................................................. 175
Adekunle M. Adesina and Ronald A. Rauch
19 Gangliocytoma and Ganglioglioma ........................................................................ 185
Carrie A. Mohila, Ronald A. Rauch, and Adekunle M. Adesina
20 Central Neurocytoma and Extraventricular Neurocytoma ................................. 195
Carrie A. Mohila, Ronald A. Rauch, and Adekunle M. Adesina
21 Papillary Glioneuronal Tumor................................................................................ 201
Marie Rivera-Zengotita, Anna Illner, and Adekunle M. Adesina
22 Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle........................... 207
Marie Rivera-Zengotita and Adekunle M. Adesina
23 Malignant Epithelioid Glioneuronal Tumor .......................................................... 211
Marie Rivera-Zengotita, Ronald A. Rauch, and Adekunle M. Adesina
24 Paraganglioma.......................................................................................................... 221
Andreana L. Rivera and Adekunle M. Adesina

Part VI Neurocutaneous Syndromes

25 Neurofibromatosis 1 ................................................................................................. 229
Tarik Tihan
26 Neurofibromatosis 2 ................................................................................................. 233
Tarik Tihan
27 Von Hippel-Lindau Disease ..................................................................................... 237
Tarik Tihan and Adekunle M. Adesina
28 Tuberous Sclerosis Complex ................................................................................... 241
Tarik Tihan and Adekunle M. Adesina
29 Lhermitte-Duclos Disease and Cowden Disease.................................................... 245
Hidehiro Takei, Hiroyoshi Suzuki, and Adekunle M. Adesina

Part VII Tumors in the Region of the Pituitary Fossa

30 Pituitary Adenomas ................................................................................................. 253
Yazgi Koy and Tarik Tihan
Contents xi

31 Rare Pituitary Tumors Other Than Adenomas ..................................................... 261

Yazgi Koy and Tarik Tihan
32 Adamantinomatous Craniopharyngioma .............................................................. 263
Tarik Tihan

Part VIII Choroid Plexus Neoplasms

33 Choroid Plexus Neoplasms ...................................................................................... 271
Christine E. Fuller

Part IX Pineal-Based Tumors

34 Pineal Parenchymal Tumors ................................................................................... 285
Tarik Tihan

Part X Germ Cell Tumors

35 Germ Cell Tumors.................................................................................................... 293
Tarik Tihan

Part XI Paraneoplastic Disorders

36 Paraneoplastic Disorders of the Central Nervous System ................................... 303
Christine E. Fuller

Part XII Tumours of the Hematopoietic System

37 Malignant Lymphoma ............................................................................................. 309
Adekunle M. Adesina
38 Histiocytic Tumors ................................................................................................... 315
Abir Mukherjee and Adekunle M. Adesina

Part XIII Interesting or Challenging Cases and Exercises

in Pattern Recognition
39 Case Studies .............................................................................................................. 323
Adekunle M. Adesina, Christine E. Fuller, and Lucy Balian Rorke-Adams

Index .................................................................................................................................. 343

Contributors

Adekunle M. Adesina, M.D., Ph.D. Department of Pathology, Texas Children’s Hospital,

Baylor College of Medicine, Houston, TX, USA
Christine E. Fuller, M.D. Division of Pathology and Laboratory Medicine, Cincinnati
Children’s Hospital Medical Center, Cincinnati, OH, USA
Jill V. Hunter, M.B.B.S. Department of Radiology, Texas Children’s Hospital, Houston, TX,
USA
Anna Illner, M.D. Department of Radiology, Texas Children’s Hospital, Houston, TX, USA
Yazgi Koy, M.D. Department of Pathology, Bagcilar Research and Training Hospital,
Bagcilar, Istanbul, Turkey
Carrie A. Mohila, M.D., Ph.D. Department of Pathology, Texas Children’s Hospital, Baylor
College of Medicine, Houston, TX, USA
Abir Mukherjee, M.D. Department of Pathology and Laboratory Medicine, Temple
University Hospital and School of Medicine, Philadelphia, PA, USA
Ronald A. Rauch, M.D. Department of Radiology, Texas Children’s Hospital, Houston, TX,
USA
Hope T. Richard, M.D., Ph.D. Department of Pathology, Virginia Commonwealth University,
Richmond, VA, USA
Andreana L. Rivera, M.D. Department of Pathology, The Methodist Hospital, Weil Cornell
Medical College, Houston, TX, USA
Marie Rivera-Zengotita, M.D. Department of Pathology, Immunology and Laboratory
Medicine, University of Florida Health College of Medicine, Gainesville, FL, USA
Lucy Balian Rorke-Adams, M.D. Department of Pathology, The Children’s Hospital of
Philadelphia, Philadelphia, PA, USA
Hiroyoshi Suzuki, M.D. Department of Pathology, Sendai Medical Center Hospital, Sendai,
Miyagi, Japan
Hidehiro Takei, M.D. Department of Pathology, The Methodist Hospital, Weil Cornell
Medical College and Baylor College of Medicine, Houston, TX, USA
Tarik Tihan, M.D., Ph.D. Department of Pathology, Neuropathology Division, San Francisco,
CA, USA

xiii
 Introduction and Overview of Brain
Tumors
Adekunle M. Adesina
1.1 Classification of Brain Tumors
• In this second edition of the Atlas of Pediatric Brain
Tumors, we have classified the tumor types based on the
2007 World Health Organization (WHO) classification of
brain tumors (Table 1.1). We have also incorporated revi-
sions in the new 2016 WHO classification of brain tumors,
with emphasis on the molecular stratification of these
tumors.
• The WHO classification assumes that the pattern of dif-
ferentiation reflects the cell of origin.
• The long-standing concept suggesting that brain tumors
arise as a result of “degeneration” of mature cell types has
been questioned.
• Current concepts suggest that all tumors arise from cancer
stem cells, which in the central nervous system (CNS)
share some common characteristics with neural progeni-
tor stem cells, including:
◦ Ability for self-renewal
◦ Ability for divergent differentiation
• The extent or pattern of differentiation reflects the nature
of activation and deregulation of specific signaling
pathways.
◦ Deregulation is often directed at genes that control
nodal points where cell growth, proliferation, and dif-
ferentiation converge with the upregulation of genes
involved in organismal survival and deregulation of
cell cycle control.
A.M. Adesina, M.D., Ph.D. (*)
Department of Pathology, Texas Children’s Hospital, Baylor
College of Medicine, One Baylor Plaza, RM 286A,
Houston, TX 77030, USA
e-mail: aadesina@bcm.tmc.edu
© Springer International Publishing AG 2016
A.M. Adesina et al. (eds.), Atlas of Pediatric Brain Tumors, DOI 10.1007/978-3-319-33432-5_1
1
1.2 Tumor Grading
• The WHO grading system is a four-tier grade system with
each increasing grade implying lesser degrees of differen-
tiation, increasing anaplasia, and increasing proliferative
potential and mitotic activity.
• The higher-grade tumors acquire aggressive characteris-
tics, which in the astrocytomas include vascular prolifera-
tion and necrosis.
• The proportion of tumor cells in the cancer stem cell pool
is lowest in the lower-grade tumors and highest in the
higher-grade tumors; it correlates with the proliferation
indices of these tumors.
• In the astrocytomas, tumors with a discrete growth pattern
rather than an infiltrative growth pattern are often ame-
nable to surgical treatment with good survival, and are
often graded as Grade I (e.g., pilocytic astrocytoma).
• Increasing anaplasia is associated with clonal evolution of
a subpopulation of cells that have acquired additional
genetic events (e.g., myc amplification), resulting in trans-
formation of classic medulloblastomas to anaplastic/
large-cell medulloblastomas.
1.3 Utility of Neuroimaging Findings
in the Interpretation of Tissue
Biopsies
• Although a tissue-based diagnosis must be made and
must stand by itself independent of neuroradiologic find-
ings, the presence of concordance or correlation between
the surgical pathology impression and the neuroradio-
logic differential diagnoses is very reassuring.
• Several neuroradiologic “rules” are especially notable:
◦ Contrast-enhancing lesions exhibiting a diffuse pattern
of growth are probably high-grade, malignant lesions.
◦ Discrete enhancing and nonenhancing lesions are sug-
gestive of low-grade or benign tumors.
1
2 A.M. Adesina

Table 1.1 Classification of tumors of the nervous system Hemangiopericytoma

Astrocytic Tumors Melanocytic lesions
Pilocytic astrocytoma Hemangioblastoma
Pilomyxoid astrocytoma Tumors of the Hematopoietic System
Pleomorphic xanthoastrocytoma Malignant lymphomas
Diffuse astrocytoma Histiocytic tumors
Anaplastic astrocytoma Germ Cell Tumors
Glioblastoma CNS germ cell tumors
Giant cell glioblastoma Familial Tumor Syndrome
Gliosarcoma Neurofibromatosis type 1
Glioblastoma cerebri Neurofibromatosis type 2
Oligodendroglial Tumors Schwannomatosis
Oligodendroglioma Von Hippel-Lindau disease and hemangioblastoma
Anaplastic oligodendroglioma Tuberous sclerosis complex and subependymal giant cell
Oligoastrocytoma astrocytoma
Anaplastic oligoastrocytoma Li-Fraumeni syndrome and TP53 germline mutations
Ependymal Tumors Cowden disease and dysplastic gangliocytoma of the cerebellum/
Lhermitte-Duclos disease
Ependymoma
Turcot syndrome
Anaplastic ependymoma
Nevoid basal cell carcinoma syndrome
Myxopapillary ependymoma
Rhabdoid tumor predisposition syndrome
Subependymoma
Tumors of the Sellar Region
Choroid Plexus Tumors
Craniopharyngioma
Choroid plexus tumors
Granular cell tumor of the neurohypophysis
Other Neuroepithelial Tumors
Pituicytoma
Astroblastoma
Spindle cell oncocytoma of the adenohypophysis
Chordoid glioma of the third ventricle
Metastatic Tumors of the CNS
Angiocentric glioma
Neuronal and Mixed Neuronal-Glial Tumors
Ganglioglioma and gangliocytoma
Desmoplastic infantile astrocytoma and ganglioglioma
Central neurocytoma and extraventricular neurocytoma ◦ Cystic mass lesions with enhancing mural nodules are
Cerebellar liponeurocytoma probably low-grade lesions, especially when located in
Papillary glioneuronal tumor (PGNT) the temporal lobe or cerebellum.
Rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) ◦ Diffusion restriction is consistent with a highly cellu-
Paraganglioma (spinal) lar tumor and raises differential diagnoses that include
Tumors of the Pineal Region
atypical teratoid rhabdoid tumor, primitive neuroecto-
Pineocytoma
dermal tumor or medulloblastoma, anaplastic ependy-
Pineal parenchymal tumor of intermediate differentiation
moma, and others.
Pineoblastoma
◦ Increased perfusion (correlating with contrast enhance-
Papillary tumor of the pineal region
ment) is usually associated with higher-grade tumors;
Embryonal Tumors
Medulloblastoma
an exception is juvenile pilocytic astrocytoma, which
CNS Primitive neuroectodermal tumor (PNET) is a low-grade tumor despite its increased perfusion.
Medulloepithelioma
Ependymoblastoma
Atypical teratoid/rhabdoid tumor 1.4 Analysis of CNS Tumors
Tumors of the Cranial Nerves
Schwannoma • Intraoperative consultation is an integral part of the man-
Neurofibroma agement of brain tumors in most centers.
Perineurioma • The goal of a gross examination is to determine how the
Malignant peripheral nerve sheath tumor (MPNST) tissue differs from normal.
Meningeal Tumors ◦ Firm to rubbery consistency often suggests a low-
Meningiomas grade glial tumor.
Mesenchymal, non-meningothelial tumors ◦ Soft, necrotic, and hemorrhagic tissue is more charac-
(continued) teristic of high-grade malignant tumors.
1 Introduction and Overview of Brain Tumors
• When histologic sections are available for review, the
following determinations must be made:
1) Is the tissue representative and diagnostic?
2) Is the lesion neoplastic or not?
3) If neoplastic, is it primary or secondary?
4) If primary, is it low-grade or high-grade?
5) Is the putative differential diagnosis corroborated by
the clinical history and preoperative neuroradiologic
findings?
• The ultimate final integrated diagnosis often has prognos-
tic implications. It must therefore be unambiguous,
because upon such a diagnosis rests the important deci-
sions regarding the utility of further attempts at surgical
resection and the appropriate use of chemotherapy and/or
radiotherapy for tumor control.
• A multidisciplinary approach to treatment decisions pro-
vides the best framework for optimal patient care.
• There is an emerging and growing list of molecular tumor
markers with potential diagnostic and prognostic utility.
Application of gene expression profiling methods and
transcriptome-wide gene sequencing have begun to define
genetic signatures that stratify many brain tumor types
into good and poor prognostic subgroups. In addition,
perturbation of critical proliferative and differentiating
pathways that drive the biology of tumor types has also
been identified, providing new targets for therapy.
Including this type of molecular information in the diag-
nosis of brain tumors is increasingly recognized, estab-
lishing new standards of practice. The new 2016 WHO
classification advocates for a combined integrated diag-
nosis based on phenotype (i.e. histologic features) and
genotype (molecular characteritics). The availability of
such data can only enhance the quality of treatment deci-
sions and is particularly critical for counseling in the set-
ting of familial tumor syndromes.
3
Suggested Reading
Ailles LE, Weissman IL. Cancer stem cells in solid tumors. Curr Opin
Biotechnol. 2007;18:460–6.
Ben-Porath I, Thomson MW, Carey VJ, Ge R, Bell GW, Regev A,
Weinberg RA. An embryonic stem cell-like gene expression signa-
ture in poorly differentiated aggressive human tumors. Nat Genet.
2008;40:499–507.
Dirks PB. Brain tumor stem cells: bringing order to the chaos of brain
cancer. J Clin Oncol. 2008;26:2916–24.
Fan X, Eberhart CG. Medulloblastoma stem cells. J Clin Oncol.
2008;26:2821–7.
Hambardzumyan D, Becher OJ, Holland EC. Cancer stem cells and
survival pathways. Cell Cycle. 2008a;7:1371–8.
Hambardzumyan D, Becher OJ, Rosenblum MK, Pandolfi PP, Manova-
Todorova K, Holland EC. PI3K pathway regulates survival of can-
cer stem cells residing in the perivascular niche following radiation
in medulloblastoma in vivo. Genes Dev. 2008b;22:436–48.
Kong DS, Kim MH, Park WY, Suh YL, Lee JI, Park K, et al. The pro-
gression of gliomas is associated with cancer stem cell phenotype.
Oncol Rep. 2008;19:639–43.
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger
D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW.
The 2016 World Health Organization classification of tumors of
the central nervous system: a summary. Acta Neuropathol.
2016;131:803–20.
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO clas-
sification of tumours of the central nervous system. Lyon:
International Agency for Research on Cancer (IARC); 2016.
Pérez Castillo A, Aguilar-Morante D, Morales-García JA, Dorado J. Cancer
stem cells and brain tumors. Clin Transl Oncol. 2008;10:262–7.
Rebetz J, Tian D, Persson A, Widegren B, Salford LG, Englund E, et al.
Glial progenitor-like phenotype in low-grade glioma and enhanced
CD133-expression and neuronal lineage differentiation potential in
high-grade glioma. PLoS One. 2008;3:e1936.
Silber J, Lim DA, Petritsch C, Persson AI, Maunakea AK, Yu M, et al.
miR-124 and miR-137 inhibit proliferation of glioblastoma multi-
forme cells and induce differentiation of brain tumor stem cells.
BMC Med. 2008;6:14.
Vigneswaran K, Neill S, Hadjipanayis CG. Beyond the World Health
Organization grading of infiltrating gliomas: advances in the molec-
ular genetics of glioma classification. Ann Transl Med. 2015;3:95.
Weller M, Weber RG, Willscher E, Riehmer V, Hentschel B, Kreuz M,
et al. Molecular classification of diffuse cerebral WHO grade II/III
gliomas using genome- and transcriptome-wide profiling improves
stratification of prognostically distinct patient groups. Acta
Neuropathol. 2015;129:679–93.
 Part I
Glial Tumors
 Pilocytic Astrocytoma and Pilomyxoid
Astrocytoma
Christine E. Fuller
2.1 Overview
• Pilocytic astrocytoma
• Pilocytic astrocytomas (PAs) are circumscribed, slow-
growing, and often cystic astrocytic neoplasms, typi-
cally with a biphasic compact and loose architecture
by histology; they correspond to World Health
Organization (WHO) grade I.
• Though most are sporadic, there is a well-established
association of PA and neurofibromatosis type 1 (NF1)
with germline mutations of the NF1 (neurofibromin)
gene. The most common CNS finding is optic nerve
involvement, particularly bilateral.
• Other synonymous terms include “optic nerve glioma,”
“cerebellar astrocytoma,” and “dorsal exophytic brain
stem glioma.”
• Pilomyxoid astrocytoma
• Pilomyxoid astrocytoma (PMA) shares some histologic
features of PA, though it tends to occur in younger chil-
dren and exhibits more aggressive clinical behavior. It
is correspondingly given a WHO grade II designation.
• PMA rarely arises within the context of NF1.
• Its histogenesis remains uncertain; theories include the
following:
– The term “tanycytoma” has been proposed by some
authors in reference to the fact that PMAs have
been shown to exhibit ultrastructural characteristics
similar to those of “tanycytes,” precursor cells
found within circumventricular organs and the floor
of the third ventricle.
C.E. Fuller, M.D. (*)
Division of Pathology and Laboratory Medicine, Cincinnati
Children’s Hospital Medical Center, 3333 Burnet Ave., MLC 1035,
Cincinnati, OH 45229-3026, USA
e-mail: christine.fuller@cchmc.org
© Springer International Publishing AG 2016
A.M. Adesina et al. (eds.), Atlas of Pediatric Brain Tumors, DOI 10.1007/978-3-319-33432-5_2
2
– PMAs may represent immature PAs, as there have
been several documented cases in which recurrent
lesions have shown features of classic PA.
2.2 Clinical Features
• Pilocytic astrocytoma
• PA is the most frequent glioma of childhood and
accounts for the vast majority of cerebellar astrocyto-
mas; it likewise represents the most common pediatric
brain tumor overall.
• Occasionally occurring in adults, most lesions come to
clinical attention within the first two decades of life.
There is no particular gender predilection.
• Clinical presentation of PA varies depending on its
localization, with the corresponding signs and symp-
toms of a slowly evolving process:
– Optic pathway lesions produce visual compromise.
– Endocrinopathies often accompany lesions of the
hypothalamus and third ventricle.
– Posterior fossa lesions often present with signs of
cerebrospinal fluid (CSF) obstruction and increase
in intracranial pressure, clumsiness, or specific
signs of brainstem dysfunction.
– Cord lesions produce localizing signs and symptoms
related to mass growth and nerve root impingement.
• Pilomyxoid astrocytoma
• PMA is much less common than PA, though the true
incidence is unclear, given that it has only recently been
well characterized from a diagnostic perspective.
• Mean age at diagnosis is 18 months (vs 5 years for PA);
it only rarely presents in adults. There is no gender
predilection.
• Patients may present with failure to thrive, developmental
delays, diplopia, and signs or symptoms related to increased
intracranial pressure and hydrocephalus. Diencephalic
syndrome may result, with extreme weight loss and
decreased fat accumulation, hyperactivity, and euphoria.
7
8 C.E. Fuller
2.3 Neuroimaging
• Pilocytic astrocytoma
• Though PAs may arise anywhere within the CNS, the
cerebellum and brainstem are the most common sites
of localization in the pediatric population, followed by
the hypothalamus or optic chiasm and optic nerve.
Less common sites include the thalamus, basal gan-
glia, cerebrum, and spinal cord.
• Intracranial lesions are frequently periventricular.
• Cerebral lesions tend to occur in an older cohort of
patients.
• Several imaging patterns have been described:
– Many show a cyst or mural nodule configuration (typ-
ically with a strongly enhancing mural nodule; the
cyst wall may or may not enhance) (Fig. 2.1A and B).
– PAs are less frequently encountered as a predomi-
nantly solid mass (Fig. 2.1C–F). PA rarely may
present as a necrotic mass with a central nonen-
hancing zone, mimicking high-grade glioma
(Fig. 2.1G).
– The cyst or mural nodule configuration is typical of
cerebellar or cerebral tumors, whereas hypotha-
lamic tumors form discrete, well-circumscribed
masses. Brainstem lesions typically manifest as a
dorsally exophytic mass (Fig. 2.1H).
– Optic nerve gliomas tend to grow as fusiform
enlargements secondary to confinement by the dura
sheath.
• PAs are hypointense on T1-weighted MRI and hyper-
intense on T2-weighted and FLAIR MR imaging
(Fig. 2.1C–E).
• The characteristic strong enhancement with contrast is
helpful in differentiating these tumors from low-grade
infiltrative gliomas, which typically do not enhance.
• They are rarely multifocal, and intralesional hemor-
rhage, calcifications, peritumoral edema, and CSF dis-
semination are uncommon.
• By MR spectroscopy, PAs most frequently show ele-
vated choline (Cho) and decreased Creatine (Cr) and
N-acetyl aspartate (NAA).
• Pilomyxoid astrocytoma
• PMA characteristically arises within the hypothalamic
or chiasmatic area; rare examples have been docu-
mented within the thalamus, temporal lobe, posterior
fossa structures, and spinal cord.
• PMAs are hypodense on nonenhanced CT scans and
heterogeneous on contrast-enhanced images.
• By MR imaging, they are solid, sometimes with cystic
or necrotic areas. Typically, they are hyperintense to
isointense on T1-weighted images and hyperintense on
T2-weighted images, hyperintense on FLAIR
sequence, and hypointense on diffusion-weighted
imaging (DWI). T2 signal abnormalities frequently
extend out into surrounding parenchyma.
• Homogeneous contrast enhancement is a consistent
finding (Fig. 2.2). Unlike PA, they do not have large
tumor cysts and are more frequently associated with
intratumoral hemorrhage or CSF dissemination.
• PMAs may show infiltration or encasement of adjacent
structures, including the circle of Willis.
• One study showed a high Cho/Cr ratio in peritumoral
regions of PMA, possibly reflecting the more aggres-
sive and infiltrative behavior of this lesion. Short echo
MR spectroscopy (MRS) showed a much higher Cho/
Cr ratio for PMA than for PA, as well as a lower Myo/
Cr ratio.
• Arterial spin labeling perfusion imaging shows prom-
ise in differentiating PMA from PA.
2.4 Pathology
• Gross pathology
• PAs tend to be well circumscribed and often cystic.
• PMAs are usually solid and may show areas of
hemorrhage.
• Intraoperative cytologic imprints and smears
• PA smear preparations contain a variably myxoid
background harboring cells with fine, elongated cell
processes and bland nuclei (Fig. 2.3A and B).
Rosenthal fibers and eosinophilic granular bodies
(EGBs) are helpful findings when present (Fig. 2.3C
and D).
• Nuclear pleomorphism, microcalcifications, perivas-
cular pseudorosettes, and hemosiderin-laden macro-
phages are infrequently present.
• PMA smear preparations may closely resemble those
of PA (Fig. 2.3E and F), but they are entirely lacking of
Rosenthal fibers and EGBs. The tumor cells typically
show slender fibrillary processes with uniformly round
nuclei; angiocentric arrangements of tumor cells may
be observed.
• Histology
• PA (WHO grade I): Although well known for their his-
tologic heterogeneity (see below), the most classic
appearance is that of a biphasic histopathology in
which compact arrangements of astrocytes with elon-
gated, hairlike “piloid” processes alternate with loose,
hypocellular microcystic zones containing cells with a
more stellate multipolar appearance (Fig. 2.4).
• Rosenthal fibers (brightly eosinophilic corkscrew-
shaped structures) (Fig. 2.5A), eosinophilic granular
bodies (EGBs) (Fig. 2.5B), and hyaline droplets may
be plentiful or rare, but are a consistent finding in PAs.
2 Pilocytic Astrocytoma and Pilomyxoid Astrocytoma
Fig. 2.1 (A) Coronal T1-weighted post-Gd MR image of cerebral pilo-
cytic astrocytoma (PA) showing a characteristic cyst with enhancing
mural nodule. (B) Some lesions are multicystic, as is this cerebellar
example (axial T1-weighted post-Gd). Note the variability of cyst wall
enhancement in (A) and (B). Images (C) through (F) demonstrate a
predominantly solid PA, with unusual localization in the insular region.
The lesion is hypointense on T1 (C), hyperintense on T2 (D) and
9
FLAIR (E), and shows only slight contrast enhancement (F). Rarely,
PA can present as a ring-enhancing lesion with central necrosis (G); this
tumor likewise has surrounding edema, and closely mimics glioblas-
toma from a radiographic perspective. (H) Sagittal T2-weighted MR
image of a dorsally exophytic brainstem PA. (B – G ) Courtesy of
Dr. Gary Tye, Virginia Commonwealth University, Richmond, VA)
10
Fig. 2.2 Axial T1-weighted post-Gd MR image of a pilomyxoid astro-
cytoma (PMA) showing a large suprasellar lesion with strong contrast
enhancement
• Spread often occurs along perivascular Virchow-Robin
and subarachnoid spaces (Fig. 2.6), the latter fre-
quently accompanied by a rich reticulin network.
• Large, multinucleated cells (“pennies on a plate”),
nuclear pleomorphism (Fig. 2.7A and B), and complex
glomeruloid vascular structures (Fig. 2.7C) may be
encountered, and are of no prognostic significance.
Infarctlike necrosis may also be seen.
• Though discrete-appearing grossly, over 50 % will
exhibit microscopically infiltrative margins (“creeping
substitution”).
• Examples that are extensively infiltrative are referred
to as the “diffuse” pattern, though they share the same
favorable prognosis as typical PAs; these most often
arise in the cerebellar hemispheres.
• Other diagnostically problematic patterns must be
considered:
– Extracellular myxoid material and perinuclear
halos can be extensive, mimicking oligodendrogli-
oma (Fig. 2.8A).
C.E. Fuller
• Vascular hyalinization and telangiectasias or angioma-
tous features are occasionally prominent enough to
suggest a diagnosis of vascular malformation.
– Perivascular pseudorosettes (mimicking ependy-
moma or angiocentric glioma) or rhythmic pali-
sades resembling spongioblastoma may be
encountered (Fig. 2.8B and C).
• Anaplastic malignant progression is extremely rare,
often associated with prior radiation therapy. Though
not yet clearly defined, these lesions should display
some degree of hypercellularity, overt nuclear atypia,
and brisk mitotic activity. Palisading necrosis may
rarely be present. Endothelial proliferation may be seen
in classic PA, especially in the cyst wall, and its pres-
ence alone does not imply anaplastic transformation.
• PMA (WHO grade II): The name “pilomyxoid astrocy-
toma” accurately portrays its histologic findings, con-
taining a monomorphous population of bipolar cells
with delicate, elongated “piloid” processes bathed by an
abundant myxoid matrix. These cell processes frequently
radiate from vessels in a pseudorosette fashion (Fig. 2.9).
• Rosenthal fibers and EGBs are absent, as is the charac-
teristic biphasic compact and loose architecture of
classic PA.
• Focal areas of necrosis, hemorrhage, or vascular pro-
liferation may be seen, and mitotic figures range from
few to quite frequent.
• “Intermediate” tumors with features of both PMA and
PA have been encountered, harboring cells with more
cytoplasm, microcysts, and thickened blood vessels.
• Occasional PMAs and intermediate tumors may
undergo maturation into PAs over time.
2.5 Electron Microscopy
• Pilocytic astrocytoma
• Ultrastructurally, the individual cells contain abundant
cytoplasmic intermediate filaments, and Rosenthal
fibers correspond to intracellular amorphous electron-
dense masses surrounded by intermediate filaments.
• Pilomyxoid astrocytoma
• Tumor cells are typically bipolar, extending elongated
thin cell processes to rest upon the basal lamina of
bloods vessels in pseudorosette-like formations
(Fig. 2.10A).
• Some cells contain abundant intermediate (glial)
filaments.
• Apical surfaces may display microvilli, blebs, and
occasional cilia.
• Vesicles and coated pits, as well as dense core granules
(Fig. 2.10B) and “synaptoid” complexes have all been
described.
2 Pilocytic Astrocytoma and Pilomyxoid Astrocytoma 11

Fig. 2.3 Smear preparations of pilocytic astrocytoma at low magnifi- bodies (D) are a helpful diagnostic feature when present. Cytologic
cation (A) and high magnification (B) showing bipolar cells with elon- preparations of PMA (E and F) show similar features to PA, but they
gated, hairlike processes. Rosenthal fibers (C) and eosinophilic granular have notably more myxoid background material
12 C.E. Fuller

Fig. 2.4 (A) Classic biphasic appearance of pilocytic astrocytoma delicate processes. (D) In more compact areas, cells tend to be elon-
(PA) with alternating compact and loose architectures. (B, C) Within gated and their delicate nature less obvious; often Rosenthal fibers are
the looser microcystic areas, myxoid material is often present, and cells seen in these areas
may take on either a bipolar or stellate appearance, bearing elongated

Fig. 2.5 (A) Rosenthal fibers may on occasion be excessive, obscuring the underlying neoplasm. (B) Eosinophilic granular bodies (EGBs) are
typically present in the less compact/microcystic areas of pilocytic astrocytoma
2 Pilocytic Astrocytoma and Pilomyxoid Astrocytoma 13

2.6 Immunohistochemistry

• Pilocytic astrocytoma
• As with other astrocytic lesions, glial fibrillary acidic
protein (GFAP) positivity can be demonstrated by
immunohistochemistry; it is typically most prominent
in the compact zones (Fig. 2.11A).
• The Ki67/MiB-1 proliferation index is low, but this
finding is not helpful in differentiating PA from low-
grade infiltrative astrocytomas.
• Absence of intratumoral anti-neurofilament-positive
processes indicative of a solid growth pattern is helpful
in differentiating PA from more diffusely infiltrative
astrocytomas (Fig. 2.11B).
• Pilomyxoid astrocytoma
Fig. 2.6 Leptomeningeal extension is not unusual for PAs, and is of no • Lesional cells are characteristically diffusely positive
prognostic significance for GFAP, S100, and vimentin (Fig. 2.11C).

Fig. 2.7 (A, B) Degenerative-type nuclear pleomorphism and multi- Glomeruloid vascular structures may likewise be present and do not
nucleated tumor giant cells are not infrequent in PA, and again are not connote malignant degeneration
prognostically relevant. Numerous EGBs may be seen (B). (C)
14
Fig. 2.8 (A) As one of the great mimickers, PAs may harbor areas
resembling oligodendroglioma, with clear zones or cytoplasm sur-
rounding rounded nuclei. (B) Perivascular pseudorosettes similar to
those of ependymomas are seen on occasion. (C) Rhythmic “spongio-
• Synaptophysin may be positive (Fig. 2.11D), though
other neuronal markers (chromogranin and neurofila-
ment) are negative. Neurofilament may on occasion
stain overrun neuritic processes, indicating infiltrative
tumor.
• Ki67/MiB-1 labeling may range from 2 % to 20 %.
2.7 Molecular Pathology
• Pilocytic astrocytoma
• Activation of the mitogen-activated protein kinase
(MAPK) pathway has emerged as a unifying molecu-
lar feature of pilocytic astrocytoma. In addition to the
aforementioned association of PA with NF1, the fol-
lowing alterations are involved in sporadic PA
tumorigenesis:
C.E. Fuller
blastic” palisades may rarely be encountered. All of these elements
should prompt a careful search for more typical histologic features of
pilocytic astrocytoma
– Approximately 70 % of PAs harbor tandem duplica-
tion at 7q34 encoding the BRAF kinase domain,
with associated KIAA1549-BRAF fusion. Multiple
fusion variants have been described, all conferring
constitutive kinase activity. KIAA1549-BRAF
fusions are significantly more frequent in cerebellar
and hypothalamic or optic pathway and brainstem
tumors than in supratentorial lesions. These fusions
do not appear to be a feature of high-grade gliomas,
though they may be found in occasional grade II
astrocytomas and glioneuronal tumors.
– BRAF V600E mutations are present in approxi-
mately 10 % of PAs and are most strongly associ-
ated with an extracerebellar location, particularly
diencephalic tumors.
– Additional alternate mechanisms for MAPK activa-
tion have been described in occasional PAs, including
Fig. 2.9 (A, B) Pilomyxoid astrocytomas are characterized by a they always lack Rosenthal fibers and EGBs, unlike PAs. Perivascular
monotonous population of cells with bland nuclei and elongated cell pseudorosettes (A, B, and D) are a frequent finding (D, Courtesy of
processes scattered amongst a background of abundant myxoid mate- Dr. Arie Perry, University of California, San Francisco)
rial. (C, D) These lesions may be of low to moderate cellularity, though

Fig. 2.10 (A) Ultrastructurally, PMAs contain cells with a bipolar morphology, with elongated, thin cell processes extending to rest upon the basal
lamina of blood vessels (corresponding to perivascular pseudorosettes by histology). (B) Dense core granules may be seen in some cases
16
Fig. 2.11 (A) PAs are diffusedly positive for glial fibrillary acidic pro-
tein (GFAP). (B) Neurofilament staining may be helpful in showing the
solid growth pattern of PA, here seen as a lack of neurofilament-stained
SRGAP3-RAF1 fusion, a number of other BRAF
fusions (involving FAM131B, RNF130, CLCN6,
MKRN1, and GNAI1), AKI-NTRK2 and NACC2-
NTRK2 fusions, and activating mutations of KRAS,
FGFR1, and PTPN11.
• Hedgehog pathway activation may be encountered in a
significant proportion of PAs arising in younger chil-
dren (<10 years of age).
• PAs have been found to lack IDH1 or IDH2 mutations,
as well as histone H3-K27M mutation.
• p16 deletion is more frequent in PAs of the brainstem
and spinal cord. Homozygous p16 deletions and het-
erozygous PTEN/10q deletions are generally limited
to PA with anaplastic features (hypercellularity, cyto-
logic atypia, and brisk mitotic activity with or without
necrosis).
C.E. Fuller
processes in the central portion of this photomicrograph. (C) PMA is
similarly diffusely positive for GFAP. (D) PMA may be similarly posi-
tive for synaptophysin
• Pilomyxoid astrocytoma
• KIAA1549-BRAF fusions have been detected in
approximately 60 % of PMA; BRAF V600E mutation
has also been described in rare examples.
• Genome-wide gene copy number analysis suggests
that PMA may represent a genetically related, more
aggressive variant of PA, with considerable overlap of
copy number alterations detected in these two entities.
• Significant differences in gene expression between
PMA and PA include overexpression (in the former) of
developmental genes H19 and DACT2, extracellular
matrix collagens (COL2A1;COL1A1), and IGF2BP3
(IMP3). Of interest, IMP3 has recently been identified
as a potential predictor of poor prognosis in pediatric
PAs or PMAs, though confirmatory studies in larger
patient cohorts are warranted.
2 Pilocytic Astrocytoma and Pilomyxoid Astrocytoma
2.8 Differential Diagnosis
• Pilocytic astrocytoma: Because it may display a variety
of histologic patterns, PA may be confused with many dif-
ferent neoplastic (and nonneoplastic) processes:
• The compact areas of PA may be indistinguishable from
areas of “piloid gliosis” containing abundant Rosenthal
fibers. Correlation with radiographic and surgical find-
ings will frequently aid in this differentiation.
• PA may resemble low-grade infiltrative astrocytoma
(or sometimes oligodendroglioma, as noted above),
particularly on limited biopsies.
• Diffuse PAs, by definition, tend to be more infiltrative,
so infiltration alone cannot be used as a differentiating
feature, but Rosenthal fibers and EGBs are reliable
clues to the diagnosis of PA in these settings. BRAF
fusion analysis also may be helpful, as most diffuse
PAs harbor KIAA1549-BRAF fusions.
• Differentiation of PAs with atypical radiographic or his-
tologic features from malignant gliomas may likewise be
challenging. Rosenthal fibers and EGBs, when present,
are helpful to support the former diagnosis. KIAA1549-
BRAF fusions likewise are frequently present in PAs,
whereas the presence of TP53 or histone H3-K27M
mutation would be supportive of malignant glioma.
• Architectural patterns such as ependymoma-like peri-
vascular pseudorosettes and rhythmic “spongioblas-
tic” palisades should always prompt a careful search
for other, more classic features of PA.
• Care should be taken in the assessment of small biop-
sies from presumed optic nerve PAs, as one may
encounter significant meningothelial hyperplasia that
could be misinterpreted as meningioma.
• Pilomyxoid astrocytoma
• Though PMA may closely resemble PA, the absence
of Rosenthal fibers, EGBs, and biphasic architecture,
as well as the presence of abundant myxoid back-
ground and angiocentric arrangements help to differ-
entiate PMA from PA.
• The perivascular pseudorosettes of PMA may lead one
to consider ependymoma, but ependymoma does not
tend to bear an abundant myxoid background, nor does
it typically show positivity for synaptophysin.
Ependymomas may uncommonly involve the hypotha-
lamic/chiasmatic region, as is typical of PMA.
2.9 Prognosis
• Pilocytic astrocytoma
• Prognosis is excellent overall, with mean progression-
free survival time of 13 years and overall survival time
of 20 years. Unfortunately, tumors arising in unfavor-
able locations may occasionally result in significant
17
morbidity and mortality despite slow growth. In these
cases, chemotherapy and gamma knife radiosurgery
may be helpful in disease stabilization.
• Recurrence is more common in chiasmatic/hypotha-
lamic lesions, these tumors being particularly difficult to
completely excise given the high morbidity and mortality
rates associated with surgery in this region. Chemotherapy
is therefore the primary treatment in these cases, with
surgery reserved for posttherapy relapses.
• Molecular pathology can influence prognosis:
– Loss of heterozygosity of 17p13 has been associ-
ated with an increased risk of recurrence in cerebel-
lar PAs.
– KIAA1549-BRAF fusion represents a significant
favorable prognostic factor when detected in incom-
pletely resected pediatric, low-grade astrocytomas
independent of age, location, or pathology (though
most represent PAs).
– In tumors with BRAF fusions, homozygous p16
deletion correlates with shorter progression-free
survival.
• Pilomyxoid astrocytoma
• PMA behaves more aggressively than PA, with a
higher rate of recurrence and shortened progression-
free and overall survivals.
• On occasion, these tumors are rapidly fatal and may
show CSF dissemination.
• Cerebellar PMAs tend to have more favorable out-
come, mainly because of the greater ease of surgical
removal in comparison with other locations.
• Immunohistochemical expression of nestin and elevated
Mib-1 labeling index have also been suggested to corre-
late with aggressiveness of PAs and PMAs.
• For either tumor type, very young children (<3 years)
generally have much worse outcomes than older children
in terms of overall survival; complete surgical resection
affords prolonged progression-free and overall survival.
Suggested Reading
Alkonyi B, Nowak J, Gnekow AK, Pietsch T, Warmuth-Metz
M. Differential imaging characteristics and dissemination potential
of pilomyxoid astrocytomas versus pilocytic astrocytomas.
Neuroradiology. 2015;57:625–38.
Bar EE, Lin A, Tihan T, Burger PC, Eberhart CG. Frequent gains at
chromosome 7q34 involving BRAF in pilocytic astrocytoma.
J Neuropathol Exp Neurol. 2008;67:878–87.
Barton VN, Donson AM, Birks DK, Kleinschmidt-DeMasters BK,
Handler MH, Foreman NK, et al. Insulin-like growth factor 2
mRNA binding protein 3 expression is an independent prognostic
factor in pediatric pilocytic and pilomyxoid astrocytoma.
J Neuropathol Exp Neurol. 2013;72:442–9.
Cin H, Meyer C, Herr R, Janzarik WG, Lambert S, Jones DT, et al.
Oncogenic FAM131B-BRAF fusion resulting from 7q34 deletion
comprises an alternative mechanism of MAPK pathway activation
in pilocytic astrocytoma. Acta Neuropathol. 2011;121:763–74.
18
Colin C, Padovani L, Chappe C, Mercurio S, Scavarda D, Loundou A,
et al. Outcome analysis of childhood pilocytic astrocytomas: a ret-
rospective study of 148 cases at a single institution. Neuropathol
Appl Neurobiol. 2013;39:693–705.
Cykowski MD, Allen RA, Kanaly AC, Fung KM, Marshall R, Perry A,
et al. The differential diagnosis of pilocytic astrocytoma with atypi-
cal features and malignant glioma: an analysis of 16 cases with
emphasis on distinguishing molecular features. J Neurooncol.
2013;115:477–86.
Forshew T, Tatevossian RG, Lawson AR, Ma J, Neale G, Ogunkolade
BW, et al. Activation of the ERK/MAPK pathway: a signature
genetic defect in posterior fossa pilocytic astrocytomas. J Pathol.
2009;218:172–81.
Fuller CE, Frankel A, Smith M, Rodziewitz G, Landas SK, Caruso R,
et al. Suprasellar monomorphous pilomyxoid neoplasm: an ultra-
structural analysis. Clin Neuropathol. 2001;20:256–62.
Hawkins C, Walker E, Mohamed N, Zhang C, Jacob K, Shirinian M,
et al. BRAF-KIAA1549 fusion predicts better clinical outcome in
pediatric low-grade astrocytoma. Clin Cancer Res. 2011;17:4790–8.
Hayashi T, Haba R, Kushida Y, Katsuki N, Shibuya S, Kadota K, et al.
Pilomyxoid astrocytoma of the pineal region: cytopathological fea-
tures and differential diagnostic considerations by intraoperative
smear preparation. Diagn Cytopathol. 2015;43:121–4.
Horbinski C, Hamilton RL, Nikiforov Y, Pollack IF. Association of
molecular alterations, including BRAF, with biology and outcome in
pilocytic astrocytomas. Acta Neuropathol (Berl). 2010;119:641–9.
Horbinski C, Nikiforova MN, Hagenkord JM, Hamilton RL, Pollack
IF. Interplay among BRAF, p16, p53, and MIB1 in pediatric low-
grade gliomas. Neuro Oncol. 2012;14:777–89.
Jacob K, Albrecht S, Sollier C, Faury D, Sader E, Montpetit A, et al.
Duplication of 7q34 is specific to juvenile pilocytic astrocytomas
and a hallmark of cerebellar and optic pathway tumours. Br
J Cancer. 2009;101:722–33.
Jeon YK, Cheon JE, Kim SK, Wang KC, Cho BK, Park SH.
Clinicopathological features and global genomic copy number altera-
tions of pilomyxoid astrocytoma in the hypothalamus/optic pathway:
comparative analysis with pilocytic astrocytoma using array-based
comparative genomic hybridization. Mod Pathol. 2008;21:1345–56.
Johnson MW, Eberhart CG, Perry A, Tihan T, Cohen KJ, Rosenblum
MK, et al. Spectrum of pilomyxoid astrocytomas: intermediate pilo-
myxoid tumors. Am J Surg Pathol. 2010;34:1783–91.
Jones DT, Kocialkowski S, Liu L, Pearson DM, Backlund LM, Ichimura
K, et al. Tandem duplication producing a novel oncogenic BRAF
fusion gene defines the majority of pilocytic astrocytomas. Cancer
Res. 2008;68:8673–7.
Jones DT, Hutter B, Jager N, Korshunov A, Kool M, Warnatz HJ, et al.
Recurrent somatic alterations of FGFR1 and NTRK2 in pilocytic
astrocytoma. Nat Genet. 2013;45:927–32.
Kleinschmidt-DeMasters BK, Donson AM, Vogel H, Foreman
NK. Pilomyxoid astrocytoma (PMA) shows significant differences
in gene expression vs. pilocytic astrocytoma (PA) and variable ten-
dency toward maturation to PA. Brain Pathol. 2015;25:429–40.
C.E. Fuller
Komotar RJ, Zacharia BE, Sughrue ME, Mocco J, Carson BS, Tihan T,
et al. Magnetic resonance imaging characteristics of pilomyxoid
astrocytoma. Neurol Res. 2008;30:945–51.
Korshunov A, Meyer J, Capper D, Christians A, Remke M, Witt H,
et al. Combined molecular analysis of BRAF and IDH1 distin-
guishes pilocytic astrocytoma from diffuse astrocytoma. Acta
Neuropathol (Berl). 2009;118:401–15.
Lin A, Rodriguez FJ, Karajannis MA, Williams SC, Legault G, Zagzag
D, et al. BRAF alterations in primary glial and glioneuronal neo-
plasms of the central nervous system with identification of 2 novel
KIAA1549:BRAF fusion variants. J Neuropathol Exp Neurol.
2012;71:66–72.
Linscott LL, Osborn AG, Blaser S, Castillo M, Hewlett RH, Wieselthaler
N, et al. Pilomyxoid astrocytoma: expanding the imaging spectrum.
AJNR Am J Neuroradiol. 2008;29:1861–6.
Nagaishi M, Yokoo H, Hirato J, Yoshimoto Y, Nakazato Y. Clinico-
pathological features of pilomyxoid astrocytomas: three case
reports. Neuropathology. 2011;31:152–7.
Okano A, Oya S, Fujisawa N, Tsuchiya T, Indo M, Nakamura T, et al.
Significance of radical resection for pilomyxoid astrocytoma of the
cerebellum: a case report and review of the literature. Childs Nerv
Syst. 2013;29:1375–9.
Pfister S, Janzarik WG, Remke M, Ernst A, Werft W, Becker N, et al.
BRAF gene duplication constitutes a mechanism of MAPK pathway
activation in low-grade astrocytomas. J Clin Invest. 2008;118:
1739–49.
Rodriguez EF, Scheithauer BW, Giannini C, Rynearson A, Cen L,
Hoesley B, et al. PI3K/AKT pathway alterations are associated with
clinically aggressive and histologically anaplastic subsets of pilo-
cytic astrocytoma. Acta Neuropathol (Berl). 2011;121:407–20.
Rush SZ, Abel TW, Valadez JG, Pearson M, Cooper MK. Activation of
the Hedgehog pathway in pilocytic astrocytomas. Neuro Oncol.
2010;12:790–8.
Schindler G, Capper D, Meyer J, Janzarik W, Omran H, Herold-Mende
C, et al. Analysis of BRAF V600E mutation in 1,320 nervous sys-
tem tumors reveals high mutation frequencies in pleomorphic xan-
thoastrocytoma, ganglioglioma and extra-cerebellar pilocytic
astrocytoma. Acta Neuropathol. 2011;121:397–405.
Sievert AJ, Jackson EM, Gai X, Hakonarson H, Judkins AR, Resnick
AC, et al. Duplication of 7q34 in pediatric low-grade astrocytomas
detected by high-density single-nucleotide polymorphism-based
genotype arrays results in a novel BRAF fusion gene. Brain Pathol.
2009;19:449–58.
Solomon DA, Wood MD, Tihan T, Bollen AW, Gupta N, Phillips JJ,
Perry A. Diffuse midline gliomas with histone H3-K27M mutation:
a series of 47 cases assessing the spectrum of morphologic variation
and associated genetic alterations. Brain Pathol. 2015. doi:10.1111/
bpa.12336.
Tihan T, Fisher PG, Kepner JL, Godfraind C, McComb RD, Goldthwaite
PT, et al. Pediatric astrocytomas with monomorphous pilomyxoid
features and a less favorable outcome. J Neuropathol Exp Neurol.
1999;58:1061–8.
 Pleomorphic Xanthoastrocytoma
Christine E. Fuller
3.1 Overview
• Pleomorphic xanthoastrocytoma (PXA) is an uncommon
astrocytic lesion that—despite its significant histologic
pleomorphism—generally behaves in a less aggressive
fashion than similarly pleomorphic infiltrative gliomas;
its behavior affords it a World Health Organization
(WHO) grade II designation.
• The vast majority are sporadic, with only rare examples
described in association with neurofibromatosis type I (NF1).
• Previously referred to as fibroxanthoma and xanthosar-
coma, PXAs have also been misclassified as forms of
giant cell glioblastoma or monstrocellular sarcoma.
• Postulated to originate from subpial astrocytes, multipo-
tential neuroectodermal precursor cells, or preexisting
hamartomatous lesions, PXAs represent distinctive astro-
cytic neoplasms with a variable degree of neuronal dif-
ferentiation demonstrable by immunohistochemistry and
electron microscopy.
3.2 Clinical Features
• Representing less than 1 % of all astrocytic tumors, PXAs
most frequently arise within the first three decades of life.
No gender predilection is apparent, and occasional cases
have been reported in the elderly.
• Given their superficial “meningocerebral” localization,
patients typically present with a history of seizures, often
of a longstanding nature.
• Headaches may also occur.
C.E. Fuller, M.D. (*)
Division of Pathology and Laboratory Medicine, Cincinnati
Children’s Hospital Medical Center, 3333 Burnet Ave., MLC 1035,
Cincinnati, OH 45229-3026, USA
e-mail: christine.fuller@cchmc.org
© Springer International Publishing AG 2016
A.M. Adesina et al. (eds.), Atlas of Pediatric Brain Tumors, DOI 10.1007/978-3-319-33432-5_3
3
3.3 Neuroimaging
• PXAs are almost always supratentorial, with a cerebral
cortical location (most commonly the temporal or parietal
lobe), frequently with involvement of the leptomeninges.
• Rare sites include the cerebellum, spinal cord, thalamus,
and cerebellopontine angle.
• 70 % arise as a cyst with a solid mural nodule, the remainder
being predominantly solid with variable small, cystic areas.
• The solid component is isodense to hypodense on CT
scans, hypointense to isointense on T1-weighted MR
imaging, and isointense to hyperintense on T2-weighted
imaging; it strongly enhances following gadolinium
administration (Fig. 3.1).
• Intratumoral hemorrhage or calcifications are uncommon;
peritumoral edema may be present, but is typically minimal.
• PXAs may rarely exhibit multifocality or leptomeningeal
dissemination.
3.4 Pathology
• Gross pathology: Operative sampling of PXAs yields
solid, firm tissue (with or without a cystic component)
with variable coloration ranging from tan to yellow, the
latter areas corresponding to xanthomatous histology.
• Leptomeninges are usually present in the sample,
incorporated into the solid portion of the tumor.
• Intraoperative cytologic imprints and smears: Cytologic
samples are polymorphous, containing cells with quite
variable cytomorphology; fibrillary astrocytic, spindled,
and giant pleomorphic forms with abundant, sometimes
vacuolated cytoplasm may all be present (Fig. 3.2).
• Eosinophilic granular bodies and scattered lympho-
cytes are commonly present.
• Rare reports of CSF cytology from PXA patients were
found to contain highly pleomorphic cells with well-
defined cytoplasm that was often vacuolated.
19
20 C.E. Fuller

Fig. 3.1 Axial MR images of a

pleomorphic xanthoastrocytoma
(PXA). (A) T2-weighted image
showing a large, solid, mildly
hyperintense, well-circumscribed
nodule with associated fluid-filled
cyst arising within the outer aspects
of the right temporoparietal region.
Peritumoral edema is fairly
prominent in this case, and there is
noticeable mass effect. (B) Axial
TI-weighted post-gadolinium
imaging shows intense
homogeneous enhancement of the
solid nodule with rim enhancement
of the cyst wall

Fig. 3.2 Intraoperative cytologic smear preparations are quite polymorphous, containing a mixture of cells with fibrillary astrocytic, spindled
morphology (A) and giant pleomorphic morphology (B)

• Histology: Although PXAs as a group are quite heteroge-
neous in their histologic appearance, several key features
are consistently present in all:
• PXAs are composed of spindle cells arranged in fas-
cicles, intersecting bundles, or a storiform pattern,
together with an admixture of variably pleomorphic
giant cells, the nuclei of which may be singular, mul-
tilobated, or multiple. Nuclear hyperchromasia is typ-
ical, and intranuclear cytoplasmic invaginations are
often present (Fig. 3.3A and B).
• Large xanthomatous cells with abundant intracytoplas-
mic lipid droplets are a helpful diagnostic feature when
present (Fig. 3.3C), though these may be quite incon-
spicuous in some cases.
• A rich reticulin network surrounds individual cells and
small cell nests (Fig. 3.3D).
• Though not a diagnostic requirement, eosinophilic gran-
ular bodies (EGBs) are almost always present (Fig. 3.3C).
• Perivascular and intratumoral collections of small
lymphocytes are also frequent (Fig. 3.3E).
• Similar to pilocytic astrocytoma and ganglion cell
tumors, PXAs (particularly those examples situated
predominantly in the subarachnoid space) display a
solid growth pattern. The interface with underlying or
surrounding brain parenchyma varies, however; infil-
trative areas resembling diffuse astrocytoma may be
encountered.
• Typical PXAs have a negligible mitotic rate and are
devoid of necrosis and vascular proliferation.
• The term “PXA with anaplastic features” is reserved
for those tumors with at least five mitoses per ten high-
power fields and/or necrosis (Fig. 3.3F).
3 Pleomorphic Xanthoastrocytoma
Fig. 3.3 PXAs are notable for their heterogeneous histologic appear-
ance. (A) They fairly consistently contain spindle cells arranged in fas-
cicles or intersecting bundles, however, together with an admixture of
pleomorphic giant cells, the nuclei of which may be singular, multilo-
bated, or multiple. (B) Nuclei are often hyperchromatic, though some
cells may exhibit nuclear features similar to those seen in ganglion
cells, containing more open chromatin and singular prominent nucleoli.
(A–C) Eosinophilic granular bodies are another consistent feature. (C)
21
Cytoplasmic vacuolization may be extensive, though it may be exceed-
ingly difficult to identify in some PXAs. (D) Verification of a rich retic-
ulin network is helpful in differentiating PXA from higher-grade
gliomas. (E) Similar to gangliogliomas, PXAs also frequently contain
interspersed collections of lymphocytes. (F) This PXA with anaplastic
features was notable for brisk mitotic activity; necrosis was present
elsewhere in the tumor
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single powerful spring, fly directly upwards for eight or ten yards, and
then proceed in a straight line. Now, if you are an expert hand, is the
moment to touch your trigger, and if you delay, be sure your shot will
fall short.
As it is attached to particular feeding grounds, and returns to them
until greatly molested, you may, by secreting yourself within shooting
distance, anticipate a good result; for even although shot at, it will
reappear several times in succession in the course of a few hours,
unless it has been wounded. The gunners in the vicinity of Boston, in
Massachusetts, who kill great numbers of these birds, on account of
the high price obtained for them in the fine market of that beautiful
and hospitable city, procure them in the following manner:—They
keep live decoy-ducks of the Mallard kind, which they take with them
in their floats or boats. On arriving at a place which they know to be
suitable, they push or haul their boat into some small nook, and
conceal it among the grass or rushes. Then they place their decoys,
one in front of their ambush, the rest on either side, each having a
line attached to one of its feet, with a stone at the other end, by
which it is kept as if riding at anchor. One of the birds is retained in
the boat, where the gunner lies concealed, and in cold weather
amply covered with thick and heavy clothing. No sooner is all in
order, than the decoy-ducks, should some wild birds appear, sound
their loud call-notes, anxious as they feel to be delivered from their
sad bondage. Should this fail to produce the desired effect of
drawing the wild ducks near, the poor bird in the boat is pinched on
the rump, when it immediately calls aloud; those at anchor respond,
and the joint clamour attracts the travellers, who now check their
onward speed, wheel several times over the spot, and at last alight.
The gunner seldom waits long for a shot, and often kills fifteen or
twenty of the Black Ducks at a single discharge of his huge piece,
which is not unfrequently charged with as much as a quarter of a
pound of powder and three quarters of a pound of shot!
The Black Ducks generally appear in the sound of Long Island in
September or October, but in very cold weather proceed southward;
while those which breed in Texas, as I have been informed, remain
there all the year. At their first arrival they betake themselves to the
fresh-water ponds, and soon become fat, when they afford excellent
eating; but when the ponds are covered with ice, and they are forced
to betake themselves to estuaries or inlets of the sea, their flesh
becomes less juicy and assumes a fishy flavour. During continued
frost they collect into larger bodies than at any other time, a flock
once alighted seeming to attract others, until at last hundreds of
them meet, especially in the dawn and towards sunset. The larger
the flock however, the more difficult it is to approach it, for many
sentinels are seen on the look-out, while the rest are asleep or
feeding along the shores. Unlike the “Sea Ducks,” this species does
not ride at anchor, as it were, during its hours of repose.
My friend, the Reverend Dr John Bachman, assures me that this
bird, which some years ago was rather scarce in South Carolina, is
now becoming quite abundant in that state, where, during autumn
and winter, it resorts to the rice fields. After feeding a few weeks on
the seeds it becomes fat, juicy, and tender. He adds that the farther
inland, the more plentifully does it occur, which may be owing to the
many steamers that ply on the rivers along the sea coast, where very
few are to be seen. They are however followed in their retreats, and
shot in great numbers, so that the markets of Charleston are now
amply supplied with them. He also informs me that he has known
hybrid broods produced by a male of this species and the common
domestic duck; and that he had three of these hybrid females, the
eggs of all of which were productive. The young birds were larger
than either of their parents, but although they laid eggs in the course
of the following spring, not one of these proved impregnated. He
further states that he procured three nests of the Dusky Duck in the
State of New York.
The young of this species, in the early part of autumn, afford
delicious eating, and, in my estimation, are much superior in this
respect to the more celebrated Canvass-back Duck. That the
species should not before now have been brought into a state of
perfect domestication, only indicates our reluctance unnecessarily to
augment the comforts which have been so bountifully accorded by
Nature to the inhabitants of our happy country. In our eastern
markets the price of these birds is from a dollar to a dollar and fifty
cents the pair. They are dearer at New Orleans, but much cheaper in
the States of Ohio and Kentucky, where they are still more abundant.
Their feathers are elastic, and as valuable as those of any other
species.
I have represented a pair of these birds procured in the full
perfection of their plumage.

Anas obscura, Lath. Synops. iii. p. 545.—Ch. Bonaparte, Synopsis of Birds

of the United States, p. 384.
Dusky Duck, Anas obscura, Wils. Amer. Ornith. vol. viii. p. 141. pl. 72. fig. 5.
Dusky Duck, Nuttall, Manual, vol. ii. p. 392.

Adult Male. Plate CCCII, Fig. 1.

Bill about the length of the head, higher than broad at the base,
depressed and widened towards the end, rounded at the tip. Upper
mandible with the dorsal line sloping and a little concave, the ridge at
the base broad and flat, towards the end broadly convex, as are the
sides, the edges soft and thin, the marginal lamellæ about forty on
each side; the unguis obovate, curved, abrupt at the end. Nasal
groove elliptical, sub-basal, filled by the soft membrane of the bill;
nostrils sub-basal, placed near the ridge, longitudinal, elliptical,
pervious. Lower mandible slightly curved upwards, flattened, with the
angle very long, narrow, and rather pointed, the lamellæ about sixty.
Head of moderate size, oblong, compressed; neck rather long and
slender; body full, depressed. Feet short, stout, placed a little behind
the centre of the body; legs bare a little above the joint; tarsus short,
a little compressed, anteriorly with small scutella, externally of which
is a series continuous with those of the outer toe, laterally and
behind with reticulated angular scales. Hind toe extremely small, with
a very narrow membrane; third toe longest, fourth a little shorter, but
longer than the second; the scutella of the second and third oblique,
of the outer transverse; the three anterior toes connected by
reticulated membranes, the outer with a thick margin, the inner with
the margin extended into a slightly lobed web. Claws small, arched,
compressed, rather obtuse, that of the middle toe much larger, with a
dilated, thin edge.
Plumage dense, soft, and elastic; on the head and neck the feathers
linear-oblong, on the other parts in general broad and rounded.
Wings of moderate breadth and length, acute; primaries narrow and
tapering, the second longest, the first very little shorter; secondaries
broad, curved inwards, the inner elongated and tapering. Tail short,
much rounded, of eighteen acute feathers, none of which are
reserved.
Bill yellowish-green, the unguis dusky. Iris dark brown. Feet orange-
red, the webs dusky. The upper part of the head is glossy brownish-
black, the feathers margined with light brown; the sides of the head
and a band over the eye are light greyish-brown, with longitudinal
dusky streaks; the middle of the neck is similar, but more dusky. The
general colour is blackish-brown, a little paler beneath, all the
feathers margined with pale reddish-brown. The wing-coverts are
greyish-dusky, with a faint tinge of green; the ends of the secondary
coverts velvet-black. Primaries and their coverts blackish-brown, with
the shafts brown; secondaries darker; the speculum is green, blue,
violet, or amethyst purple, according to the light in which it is viewed,
bounded by velvet-black, the feathers also tipped with a narrow line
of white. The whole under surface of the wing, and the axillaries,
white.
Length to end of tail 24 1/2 inches, to end of claws 26; extent of
wings 38 1/2; bill 2 4/12 along the back; wing from flexure 11 1/2; tail
4 4/12; tarsus 1 6 1/2 /12; middle toe 2 3/12, its claw 4/12; first toe 5/12, its
claw 2/12. Weight 3 lb.

Adult Female. Plate CCCII. Fig. 2.

The female, which is somewhat smaller, resembles the male in
colour, but is more brown, and has the speculum of the same tints,
but without the white terminal line.
Length to end of tail 22 inches, to end of wings 21 1/4, to end of
claws 22; wing from flexure 10 1/2; extent of wings 34 1/4; tarsus 2,
middle toe and claw 2 1/2; hind toe and claw 5/12.

In this species, the number of feathers in the tail is eighteen,

although it has been represented as sixteen. In form and proportions
the Dusky Duck is very closely allied to the Mallard. The following
account of the digestive and respiratory organs is obtained from the
examination of an adult male.
On the upper mandible are 43 lamellæ; on the lower, 85 in the upper,
and 56 in the lower series. The tongue is 1 1/12 inch long, with the
sides parallel and furnished with a double row of filaments,
numerous small conical papillæ at the base, a median groove on the
upper surface, and a thin rounded appendage, a twelfth and a half in
length at the tip. The aperture of the glottis is 7 1/2/12 long, with very
numerous minute papillæ behind. The œsophagus 12 inches long, of
a uniform diameter of 4/12, until near the lower part of the neck,
where it enlarges to 8/12, again contracts as it enters the thorax,
ending in the proventriculus, which is 1 1/4 long, with numerous
oblong glandules, about a twelfth in length. Gizzard obliquely
elliptical, 2 1/4 inches across, 1 8/12 in length, its lateral muscles
extremely large, the left 10/12 in thickness, the right 9/12; their tendons
large and strong; the lower muscle moderately thick; the cuticular
lining firm and rugous, the grinding surfaces nearly smooth. The
intestine, which is 5 feet 7 1/2 inches long, is slender and nearly
uniform in diameter, measuring 4/12 across in the duodenal portion,
3/ in the rest of its extent; the rectum 3 1/2 inches long, dilated into
12
a globular cloaca 1 inch in length, and of nearly the same diameter.
The cæca are 6 1/4 long, 1 1/2/12 in diameter for 2 inches of their
length, enlarged to 3/12 in the rest of their extent, and terminating in
an obtuse extremity.
The trachea, moderately extended, is 10 inches long. Its lateral or
contractor muscles are strong, and it is furnished with a pair of
cleido-tracheals, and a pair of sterno-tracheals. The number of rings
is 136, besides 12 united rings forming a large inferior larynx, which
has a transversely oblong bony expansion, forming on the left side a
bulging and rounded sac. There are 28 bronchial half rings on the
right side, 26 on the left.
 BARTRAMIAN SANDPIPER.

Totanus Bartramius, Temm.

PLATE CCCIII. Male and Female.

The Bartramian Sandpiper is the most truly terrestrial of its tribe with
which I am acquainted. It is even more inclined, at all seasons, to
keep away from the water, than the Kildeer Plover, which may often
be seen wading in shallow pools, or searching along the sandy or
muddy margins of the shores of the sea, or of fresh-water lakes and
streams. Although not unfrequently met with in the vicinity of such
places, it never ventures to wade into them; and yet the form and
length of its legs and feet would naturally induce a person not
acquainted with its habits to consider it as a wading bird.
The dry upland plains of those sections of Louisiana called
Opellousas and Attacapas, are amply peopled with this species in
early spring, as well as in autumn. They arrive there from the vast
prairies of Texas and Mexico, where they spend the winter, in the
beginning of March, or about the period of the first appearance of the
Martins, Hirundo purpurea, and return about the first of August. They
are equally abundant on all the western prairies on either side of the
Missouri, where, however, they arrive about a month later than in
Louisiana, whence they disperse over the United States, reaching
the middle districts early in May, and the State of Maine by the
middle of that month, or about the same period at which they are
seen in Indiana, Kentucky, and Ohio. Some proceed as far north as
the plains adjoining the Saskatchewan River, where Dr Richardson
met with this species in the month of May.
It has been supposed that the Bartramian Sandpiper never forms
large flocks, but this is not correct, for in the neighbourhood of New
Orleans, where it is called the “Papabote,” it usually arrives in great
bands in spring, and is met with on the open plains and large grassy
savannahs, where it generally remains about two weeks, though
sometimes individuals may be seen as late as the 15th of May. I
have observed the same circumstances on our western prairies, but
have thought that they were afterwards obliged to separate into
small flocks, or even into pairs, as soon as they are ready to seek
proper places for breeding in, for I have seldom found more than two
pairs with nests or young in the same field or piece of ground. On
their first arrival, they are generally thin, but on their return
southward, in the beginning of August, when they tarry in Louisiana
until the first of October, they are fat and juicy. I have observed, that
in spring, when they are poor, they are usually much less shy than in
autumn, when they are exceedingly wary and difficult of approach;
but this general observation is not without exceptions, and the
difference, I think, depends on the nature of the localities in which
they happen to be found at either period. When on newly ploughed
fields, which they are fond of frequenting, they see a person at a
greater distance than when they are searching for food among the
slender grasses of the plains. I have also thought that the size of the
flocks may depend upon similar contingencies, for this bird is by no
means fond of the society of man.
Like the Spotted Sandpiper, Totanus macularius, they not
unfrequently alight on fences, trees, and out-houses; but whether in
such situations or on the ground, they seldom settle without raising
both wings upright to their full extent, and uttering their loud and
prolonged, but pleasing notes. They run with great activity, stop
suddenly, and vibrate their body once or twice. When earnestly
followed by the sportsman, they lower their heads in the manner of
Wilson’s Plover, and the species called the Piping, and run off
rapidly, or squat, according to the urgency of the occasion. At other
times, they partially extend their wings, run a few steps as if about to
fly, and then cunningly move off sideways, and conceal themselves
among the grass, or behind a clod. You are not unfrequently
rendered aware of your being within sight of them, by unexpectedly
hearing their plaintive and mellow notes, a circumstance, however,
which I always concluded to be indicative of the wariness of their
disposition, for although you have just heard those well-known cries,
yet, on searching for the bird, you nowhere see it, for the cunning
creature has slipped away and hid itself. When wounded in the wing,
they run to a great distance, and are rarely found.
Like all experienced travellers, they appear to accommodate
themselves to circumstances as regards their food, for in Louisiana,
they feed on cantharides and other coleopterous insects; in
Massachusetts on grasshoppers, on which my friend Nuttall says,
they soon grow very fat; in the Carolinas on crickets and other
insects, as well as the seeds of the crab-grass, Digitaria sanguinaria;
and in the barrens of Kentucky they often pick the strawberries.
Those which feed much on cantharides, require to be very carefully
cleaned, otherwise persons eating them are liable to suffer severely.
Several gentlemen of New Orleans have assured me, that they have
seen persons at dinner obliged to leave the room at once, under
such circumstances, which cannot well be described here. When
flavoured with the ripe strawberries, on which they have fed, their
flesh is truly delicious.
This species performs its migrations by night as well as by day. Its
flight is rather swift and well sustained. While travelling, it generally
flies so high as to be beyond reach of the gun: but if the weather be
cloudy, or if it blow hard, it flies lower, and may easily be shot. It
generally proceeds in straggling bands, and moves along with
continuous easy beats of the wings, but sails, as it were, when about
to alight, as well as during the love season.
As long ago as 1805 and 1806, I observed this species breeding in
the meadows and green-fields of my plantation of Millgrove, near the
banks of Perkioming Creek. Since then, I have known of its rearing
broods in different parts of Pennsylvania, in the State of New York,
and in various districts to the eastward as far as the confines of
Maine; but I did not find it in Newfoundland or Labrador; and I have
reason to believe that it does not breed to the south of Maryland.
My friend, the Rev. Dr Bachman, has informed me that the
Bartramian Sandpiper makes its appearance in South Carolina about
the 15th of July, the hottest period of the year, in considerable
numbers, betakes itself at once to the high grassy lands, and there
remains about a month. He considers it to be then on its return from
the north, and states that it is very fat and affords delicious food. His
manner of shooting them is, to ride in a chair or gig over the fields
which they frequent, or along the roads in their neighbourhood, by
which means they can be approached near enough to enable the
sportsman to shoot with almost a certainty of success, as the bird
rises out of the grass. If one attempts to get near them on foot, they
rise at too great a distance, then sweep in circles over the spot, and
alight a considerable way off. They are seldom met with there in
flocks of more than four or five individuals.
I have found the eggs of this bird laid on the bare earth, in a hollow
scooped out to the depth of about an inch and a half, near the roots
of a tuft of rank grass, in the middle of a meadow, and seen some
nests of the same species formed of loosely arranged grasses, and
placed almost beneath low bushes growing on poor elevated ridges,
furnished with a scanty vegetation. I have also heard my esteemed
young friend, John Trudeau, state that he had discovered one on a
high part of the bank of the Delaware River. When disturbed while on
its nest, but unobserved, it runs thirty or forty yards, and then flies off
as if severely wounded. Should it have young, its attempts to decoy
you away are quite enough to induce you to desist from harassing it.
The eggs measure an inch and five and a half eighths, by an inch
and a quarter in their greatest breadth. In form they resemble those
of Totanus macularius, being broadly rounded at one end, and rather
pointed at the other; their surface smooth; their ground colour dull
greyish-yellow, with numerous spots of light purple and reddish-
brown. They are placed in the nest in the same manner as those of
the Spotted Sandpiper, that is, with the smaller ends together, which
is also the case with those of the Tell-tale Godwit, Wilson’s Plover,
and the Kildeer Plover. The young, which run about immediately
after exclusion, grow rapidly, and in about a month are able to use
their wings, after which, they and their parents gradually, and
according to the temperature of the season, move southward.
In Massachusetts, and to the eastward of that state, this species is
best known by the name of “Upland Plover,” and in some other
districts it is named the Field Plover. The drawing from which the
plate was engraved was taken from individuals shot near Bayou
Sara, in the State of Mississippi.

Totanus Bartramius, Ch. Bonap., Synopsis of Birds of the United States, p.

262.
Tringa Bartramia, Wils. Amer. Ornith. vol. vii. p. 63. pl. 59. fig. 2.
Bartramian Tatler, Nuttall, Manual, vol. ii. p. 169.
Totanus Bartramius, Richards. and Swains. Fauna Bor. Amer. vol. ii. p. 391.

Adult Male. Plate CCCIII. Fig. 1.

Bill a little longer than the head, slender, straight, slightly defected at
the end. Upper mandible with the dorsal line straight, the ridge
convex, the sides grooved beyond the middle, afterwards convex,
the edges inflected, the tips a little deflected, and tapering to an
obtuse point. Nostrils sub-basal, lateral, linear, pervious, nearer the
edge than the dorsal line. Lower mandible, with the angle very
narrow and elongated, beyond it the outline slightly convex, the sides
sloping outwards and concave until the middle, afterwards flattened,
the edges sharp, the point very narrow.
Head rather small, convex above, compressed. Neck of moderate
length, slender. Body rather slender. Feet long and slender; tibia
bare for about half its length, scutellate before and behind; tarsus
long, slender, having before and behind numerous scutella, the
narrow lateral spaces with very small oblong scales. Toes slender,
the first very short, the second much shorter than the fourth, the third
and fourth connected at the base by a web; the scutella numerous;
claws small, compressed, slightly arched, rather blunt.
Plumage soft, on the neck and lower parts, blended; on the upper
rather distinct. Wings rather long, acute, narrow; primaries tapering,
and rounded, the first longest, the second a little shorter, the rest
rapidly graduated; secondaries obliquely rounded, the inner
elongated and tapering. Tail of moderate length, much rounded, of
twelve rather narrow feathers.
Bill yellowish-green, the tip dusky, the edges towards the base
yellow. Iris dark hazel. Legs and tarsi light yellowish-grey, toes rather
darker, claws brownish-black. Upper part of the head dark brown,
with a median pale yellowish-brown line, the margins of the feathers
also of that colour, which prevails along the sides of the head and
the back of the neck, which are streaked with dusky; the eye
surrounded with yellowish-white. Throat yellowish white, without
spots; fore-part and sides of the neck, with a portion of the breast
and sides of the body, cream-coloured, with dusky lines, which
gradually become arrow-shaped on the breast, forming a double
transverse band; the feathers on the sides barred; the rest of the
lower parts yellowish-white, the lower tail-coverts rich cream-
coloured. Axillar feathers and lower wing-coverts white, banded with
brownish-black. On the upper parts the feathers are dark brown,
glossed with green, with rich cream-coloured margins; the rump
darker. On the margins of the scapulars, within the pale edge, is a
series of dusky spots, which towards the end become continuous.
Alula, primary coverts, and primary quills, blackish-brown, the inner
webs crossed by white bands, until about an inch from the end, the
shaft of the first quill white, those of the rest dusky. Secondaries
greyish-brown, their outer margins pale brown, with dusky spots; the
inner darker. The two middle feathers of the tail are dark olive, tinged
with grey, transversely barred with black, the last bar arrow-shaped,
the margins light cream-colour: the next feather on each side lighter,
and tinged with yellowish-red; the rest gradually lighter, the outer
white, all barred with black.
Length to end of tail 12 1/2 inches, to end of wings 11 1/8, to end of
claws 13 1/2; extent of wings 22; wing from flexure 7; tail 3 3/4; bare
part of tibia 9/10; tarsus 1 1 1/2/12, first toe 4/12, its claw 1 1/2/12; middle-
toe 1, its claw 2 1/4/12; bill along the ridge 1 2/12; along the edge of
lower mandible 1 3/12. Weight 6 oz.
Female. Plate CCCIII. Fig. 2.
The female is a little larger, and weighs 7 oz., but resembles the
male in colour. The individual of which the weight is here given was
very fat, but I have never met with any that weighed three-fourths of
a pound, as described by Wilson!
Length to end of tail 13 inches, to end of claws 14, extent of wings
22 3/4.

In an adult bird of this species, the tongue measures seven-twelfths

of an inch in length, and is sagittate at the base, with conical papillæ,
of which the outermost is much larger, then contracted, being deeper
than broad, and tapering to a very acute compressed point. Aperture
of the glottis 2/12 long, with numerous papillæ behind, the middle two
largest. The œsophagus is 5 1/4 inches long, of uniform diameter,
measuring about 3/12 across, and passing along the right side of the
neck, along with the trachea. Proventriculus oblong, 8/12 in diameter,
its glandules extremely numerous, oblong, half a twelfth in length.
The stomach is a strong gizzard of an oblong form; an inch and a
twelfth long, nine-twelfths in breadth, its lateral muscles of moderate
thickness, the right 2 1/2/12, the left 5 1/2/12, the central tendons oblong,
5/ in diameter. The cuticular lining is tough, of moderate thickness,
12
longitudinally rugous, the grinding plates scarcely thicker than the
rest. The intestine is 18 inches long, its diameter generally 3 1/2/12.
The rectum 2 1/4 inches long; the cæca 2 2/12, very slender, their
greatest diameter being only 1 1/2/12; the cloaca globular, about 1/2
inch in diameter. The stomach was filled with remains of
grasshoppers, of a deep red colour, with which the inner coat was
tinged, together with the head of a Libellula. No gravel or other hard
substances.
The trachea moderately extended is 3 10/12 inches long, its
1/ 1/
transverse diameter 2 2/12, diminishing to 1 /12. The rings are
2

unossified and extremely thin, 105 in number; the contractor or

lateral muscles feeble; the inferior larynx simple, with a single pair of
tracheali-bronchiales, and the usual sterno-tracheales; the bronchi of
about 15 half-rings.
This individual presented a very remarkable accumulation of fat over
the abdominal and pectoral muscles, and especially about the
furcula.
 TURNSTONE.

Strepsilas Interpres, Illiger.

PLATE CCCIV. Adult in Summer and Winter.

This bird, which, in its full vernal dress, is one of the most beautiful of
its family, is found along the southern coasts of the United States
during winter, from North Carolina to the mouth of the Sabine River,
in considerable numbers, although perhaps as many travel at that
season into Texas and Mexico, where I observed it on its journey
eastward, from the beginning of April to the end of May 1837. I
procured many specimens in the course of my rambles along the
shores of the Florida Keys, and in the neighbourhood of St
Augustine, and have met with it in May and June, as well as in
September and October, in almost every part of our maritime shores,
from Maine to Maryland. On the coast of Labrador I looked for it in
vain, although Dr Richardson mentions their arrival at their
breeding quarters on the shores of Hudson’s Bay and the Arctic Sea
up to the seventy-fifth parallel.
In spring the Turnstone is rarely met with in flocks exceeding five or
six individuals, but often associates with other species, such as the
Knot, the Red-backed Sandpiper, and the Tringa subarquata.
Towards the end of autumn, however, they collect into large flocks,
and so continue during the winter. I have never seen it on the
margins of rivers or lakes, but always on the shores of the sea,
although it prefers those of the extensive inlets so numerous on our
coasts. At times it rambles to considerable distances from the beach,
for I have found it on rocky islands thirty miles from the mainland;
and on two occasions, whilst crossing the Atlantic, I saw several
flocks near the Great Banks flying swiftly, and rather close to the
water around the ships, after which they shot off toward the south-
west, and in a few minutes were out of sight. It seems to be a hardy
bird, for some of them remain in our Eastern Districts until severe
frost prevails. Having seen some, in the beginning of June, and in
superb plumage, on the high grounds of the Island of Grand
Mannan, in the Bay of Fundy, I supposed that they bred there,
although none of my party succeeded in discovering their nests.
Indeed the young, as I have been informed, are obtained there, and
along the coast of Maine, in the latter part of July.
I have found this bird much more shy when in company with other
species than when in flocks by itself, when it appears to suspect no
danger from man. Many instances of this seeming inattention have
occurred to me, among others the following:—When I was on the
island of Galveston in Texas, my friend Edward Harris, my son,
and some others of our party, had shot four deer, which the sailors
had brought to our little camp near the shore. Feeling myself rather
fatigued, I did not return to the bushes with the rest, who went in
search of more venison for our numerous crew, but proposed, with
the assistance of one of the sailors, to skin the deer. After each
animal was stripped of its hide, and deprived of its head and feet,
which were thrown away, the sailor and I took it to the water and
washed it. To my surprise, I observed four Turnstones directly in our
way to the water. They merely ran to a little distance out of our
course, and on our returning, came back immediately to the same
place; this they did four different times, and, after we were done,
they remained busily engaged in searching for food. None of them
was more than fifteen or twenty yards distant, and I was delighted to
see the ingenuity with which they turned over the oyster-shells, clods
of mud, and other small bodies left exposed by the retiring tide.
Whenever the object was not too large, the bird bent its legs to half
their length, placed its bill beneath it, and with a sudden quick jerk of
the head pushed it off, when it quickly picked up the food which was
thus exposed to view, and walked deliberately to the next shell to
perform the same operation. In several instances, when the clusters
of oyster-shells or clods of mud were too heavy to be removed in the
ordinary way, they would use not only the bill and head, but also the
breast, pushing the object with all their strength, and reminding me
of the labour which I have undergone in turning over a large turtle.
Among the sea-weeds that had been cast on the shore, they used
only the bill, tossing the garbage from side to side, with a dexterity
extremely pleasant to behold. In this manner, I saw these four
Turnstones examine almost every part of the shore along a space of
from thirty to forty yards; after which I drove them away, that our
hunters might not kill them on their return.
On another occasion, when in company with Mr Harris, and on the
same island I witnessed the same pleasing proceeding, several
Turnstones being engaged in searching for food in precisely the
same manner. At other times, and especially when in the
neighbourhood of St Augustine, in East Florida, I used to amuse
myself with watching these birds on the racoon-oyster banks, using
my glass for the purpose. I observed that they would search for such
oysters as had been killed by the heat of the sun, and pick out their
flesh precisely in the manner of our Common Oyster-catcher,
Hæmatopus palliatus, while they would strike at such small bivalves
as had thin shells, and break them, as I afterwards ascertained, by
walking to the spot. While on the Florida coast, near Cape Sable, I
shot one in the month of May, that had its stomach filled with those
beautiful shells, which, on account of their resemblance to grains of
rice, are commonly named rice-shells.
I have always looked upon the Turnstone, while at its avocations, as
a species very nearly allied to the Oyster-catcher; and, although it
certainly differs in some particulars, were I to place it in a position
determined by its affinities, I should remove it at once from the
Tringa family. Its mode of searching for food around pebbles and
other objects, the comparative strength of its legs, its retiring
disposition, and its loud whistling notes while on wing, will, I think,
prove at some period that what I have ventured to advance may be
in accordance with the only true system, by which I mean Nature’s
own system, could one be so fortunate as to understand it.
While this species remains in the United States, although its
residence is protracted to many months, very few individuals are met
with in as complete plumage as the one represented in my plate with
the wings fully extended; for out of a vast number of specimens
procured from the beginning of March to the end of May, or from
August to May, I have scarcely found two to correspond precisely in
their markings. For this reason, no doubt exists in my mind that this
species, as well as the Knot and several others, loses its rich
summer plumage soon after the breeding season, when the oldest
become scarcely distinguishable from the young. In the spring
months, however, I have observed that they gradually improve in
beauty, and acquire full-coloured feathers in patches on the upper
and lower surfaces of the body, in the same manner as the Knot, the
Red-breasted Snipe, the Godwits, and several other species.
According to Mr Hewitson, the eggs are four in number, rather
suddenly pointed towards the smaller end, generally an inch and four
and a half eighths in length, an inch and one and a half eighths in
their greatest breadth, their ground colour pale yellowish-green,
marked with irregular patches and streaks of brownish-red, and a
few lines of black.
My drawing of the Turnstones represented in the plate was made at
Philadelphia, in the end of May 1824; and the beautiful specimen
exhibited in the act of flying, I procured near Camden, while in the
agreeable company of my talented friend Le Sueur, who, alas! is
now no more.
I have not observed any remarkable difference in the plumage of the
sexes at any season of the year. The males I have generally found to
be somewhat larger than the females, which, as is well known, is not
the case in the Tringa family.
My worthy friend, Dr Bachman, once had a bird of this species alive.
It had recovered from a slight wound in the wing, when he presented
it to a lady, a friend of his and mine, who, fed it on boiled rice, and
bread soaked in milk, of both of which it was very fond. It continued
in a state of captivity upwards of a year, but was at last killed by
accident. It had become perfectly gentle, would eat from the hand of
its kind mistress, frequently bathed in a basin placed near it for the
purpose, and never attempted to escape, although left quite at liberty
to do so.

Tringa interpres, Linn. Syst. Nat. vol i. p. 248.—Lath. Ind. Orn. vol. ii. p.
738.
Tringa morinella, Linn. Syst. Nat. vol. i. p. 249.
Turnstone, Tringa interpres, Wils. Amer. Ornith. vol. vii. p. 32. pl. 57. fig 1.
Strepsilas collaris, Temm. Man. d’Ornith, part ii. p. 553.
Strepsilas Interpres, Ch. Bonap. Synopsis of Birds of the United States,
299.
Turnstone or Sea-dotterel, Nuttall, Manual, vol. ii. p. 30.

Adult Male in Summer. Plate CCCIV. Fig. 1.

Bill a little shorter than the head, rather stout, compressed, tapering,
straightish, being recurvate in a slight degree. Upper mandible with
the dorsal line very slightly concave, the nasal groove extending to
the middle, the sides beyond it sloping, the tip depressed and
blunted. Nostrils sub-basal linear-oblong, pervious. Lower mandible
with the angle short, the dorsal line ascending and slightly convex,
the sides convex, the edges sharp, the tip depressed and blunted.
Head small, ovate; eyes of moderate size. Neck of ordinary length.
Body rather full. Feet of moderate length, stout; tibia bare at the
lower part, and covered with reticulated scales; tarsus roundish, with
numerous broad anterior scutella; toes four, the first very small, and
placed higher than the rest; the anterior toes free to the base,
distinctly margined on both edges, the inner toe a little shorter than
the outer, the third or middle toe considerably longer; claws rather
small, arcuate, compressed, blunted.
Plumage full, soft, rather dense, and glossy; feathers on the hind
neck blended, and rather narrow, on the other parts ovate. Wings
long, pointed, of moderate breadth: primaries with strong shafts,
rather broad, narrowed towards the end, the first longest, the rest
rapidly decreasing; outer secondaries incurved, obliquely rounded;
inner elongated, one of them extending to half an inch of the tip of
the longest primary, when the wing is closed. Tail rather short,
slightly rounded, of twelve moderately broad, rounded feathers.
Bill black. Iris hazel. Feet deep orange red, claws black. Plumage
variegated with white, black, brown, and red. Upper parts of the head
and nape streaked with black and reddish-white; a broad band of
white crosses the forehead, passes over the eyes, and down the
sides of the neck, the hind-part of which is reddish-white faintly
mottled with dusky; a frontal band of black curves downwards before
the eye, enclosing a white patch on the lore, and meeting another
black band glossed with blue, which proceeds down the neck, from
the base of the lower mandible, enlarging behind the ear, covering
the whole anterior part of the neck, and passing along the shoulder
over the scapulars; the throat, hind part of the back, the outer
scapulars, upper tail-coverts, and the under parts of the body and
wings, white. Anterior smaller wing-coverts dusky, the rest bright