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Textbook Bone 2Nd Edition G Petur Nielsen Ebook All Chapter PDF
Textbook Bone 2Nd Edition G Petur Nielsen Ebook All Chapter PDF
Petur Nielsen
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SECOND EDITION
NIELSEN | ROSENBERG
DESHPANDE • HORNICEK • KATTAPURAM • ROSENTHAL
ii
SECOND EDITION
G. Petur Nielsen, MD
Pathologist, Department of Pathology
Director of Electron Microscopy
Director of Bone & Soft Tissue Pathology
Massachusetts General Hospital
Professor of Pathology
Harvard Medical School
Boston, Massachusetts
Andrew E. Rosenberg, MD
Professor and Vice Chair
Director of Bone & Soft Tissue Pathology
Department of Pathology
Miller School of Medicine
University of Miami
Miami, Florida
iii
1600 John F. Kennedy Blvd.
Ste 1800
Philadelphia, PA 19103-2899
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permissions.
This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be
noted herein).
Notices
Knowledge and best practice in this field are constantly changing. As new research and
experience broaden our understanding, changes in research methods, professional practices,
or medical treatment may become necessary.
Practitioners and researchers must always rely on their own experience and knowledge in
evaluating and using any information, methods, compounds, or experiments described
herein. In using such information or methods they should be mindful of their own safety
and the safety of others, including parties for whom they have a professional responsibility.
With respect to any drug or pharmaceutical products identified, readers are advised to check
the most current information provided (i) on procedures featured or (ii) by the manufacturer
of each product to be administered, to verify the recommended dose or formula, the
method and duration of administration, and contraindications. It is the responsibility of
practitioners, relying on their own experience and knowledge of their patients, to make
diagnoses, to determine dosages and the best treatment for each individual patient, and to
take all appropriate safety precautions.
To the fullest extent of the law, neither the Publisher nor the authors, contributors, or
editors, assume any liability for any injury and/or damage to persons or property as a matter
of products liability, negligence or otherwise, or from any use or operation of any methods,
products, instructions, or ideas contained in the material herein.
iv
Dedications
To my wife and family.
GPN
v
vi
Preface
The pathology of the skeleton is complex and is the morphologic expression of a broad spectrum of
diseases, including those caused by genetic (sporadic and inherited), malformative, inflammatory, metabolic,
circulatory, traumatic, iatrogenic, and neoplastic disorders. Bone tumors, including both neoplasms
and various conditions that may simulate them, are the focus of our book. This topic is one of the most
challenging areas in surgical pathology for several reasons: Bone tumors are uncommon, making it difficult
to acquire the necessary experience with their histological variants and mimics; the correct diagnosis usually
requires the careful integration of radiological imaging studies and clinical findings; the implications of a
diagnosis on a patient can be life changing; and medical schools and pathology training programs often have
insufficient expertise to provide medical students and young pathologists with the skills needed to diagnose
these lesions accurately and precisely.
This book reflects the philosophy and high standards practiced by the truly multidisciplinary team of
physicians at the Massachusetts General Hospital and University of Miami, who have diagnosed and surgically
treated tens of thousands of patients with bone tumors for many decades. Also important to acknowledge
are the contributions of the many fellows and residents who participated in the efforts of patient care.
The authors are subspecialized physicians who have dedicated their professional lives to the diagnosis and
surgical management of bone tumors. As a result, the figures include beautiful and classic examples and
unusual variants of many of the diseases discussed and are the product of painstaking correlations between
the clinical, imaging, macroscopic, histological, immunohistochemical, and molecular characteristics of bone
tumors. The text synthesizes the literature and our combined extensive experience, and the images have
been selectively culled from the patient files of the Massachusetts General Hospital, the University of Miami
Miller School of Medicine, and the private consultations of the authors. The book is constructed in a thematic
format with sections representing groups of related diseases and the chapters discussing individual entities
and their differential diagnosis.
Accordingly, this textbook serves as an excellent resource for medical students, residents, fellows, and
practicing physicians in the disciplines of pathology, radiology, and orthopedics. Medical and radiation
oncologists who treat bone tumors will also find it valuable. Our opportunity to participate in the care
of patients with bone tumors has been our call and honor, and we hope to do it justice by sharing our
experience with the medical community—our goal is to enhance diagnostic accuracy and to provide the
biological basis for optimal treatment.
G. Petur Nielsen, MD
Pathologist, Department of Pathology
Director of Electron Microscopy
Director of Bone & Soft Tissue Pathology
Massachusetts General Hospital
Professor of Pathology
Harvard Medical School
Boston, Massachusetts
Andrew E. Rosenberg, MD
Professor and Vice Chair
Director of Bone & Soft Tissue Pathology
Department of Pathology
Miller School of Medicine
University of Miami Hospital
Miami, Florida
vii
viii
Acknowledgments
Text Editors
Arthur G. Gelsinger, MA
Nina I. Bennett, BA
Lisa A. Gervais, BS
Karen E. Concannon, MA, PhD
Matt W. Hoecherl, BS
Megg Morin, BA
Image Editors
Jeffrey J. Marmorstone, BS
Lisa A. M. Steadman, BS
Illustrations
Laura C. Sesto, MA
Richard Coombs, MS
Lane R. Bennion, MS
Lead Editor
Terry W. Ferrell, MS
Production Coordinators
Angela M. G. Terry, BA
Rebecca L. Bluth, BA
Emily C. Fassett, BA
ix
x
Sections
SECTION 1: Benign Bone-Forming Tumors
xi
TABLE OF CONTENTS
xii
TABLE OF CONTENTS
400 Leiomyosarcoma
SECTION 9: GIANT CELL-RICH TUMORS G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
256 Giant Cell Tumor 404 Myoepithelioma
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
270 Brown Tumor 410 Schwannoma
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
274 Giant Cell Reparative Granuloma 414 Myxopapillary Ependymoma
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD Vikram Deshpande, MD, G. Petur Nielsen, MD, and
Andrew E. Rosenberg, MD
SECTION 10: CYSTIC LESIONS OF BONE 416 Phosphaturic Mesenchymal Tumor
282 Intraosseous Ganglion G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
286 Unicameral Bone Cyst SECTION 14: METASTATIC TUMORS
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD 420 Metastatic Tumors
294 Aneurysmal Bone Cyst Vikram Deshpande, MD, Andrew E. Rosenberg, MD, and
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD G. Petur Nielsen, MD
306 Epidermoid Inclusion Cyst
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD SECTION 15: BONE TUMOR MIMICS
428 Bizarre Parosteal Osteochondromatous
SECTION 11: VASCULAR TUMORS
Proliferation and Related Lesions
310 Conventional Hemangioma G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD 434 Melorheostosis
318 Lymphangioma/Lymphangiomatosis G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD 438 Amyloidoma
320 Epithelioid Hemangioma G. Petur Nielsen, MD, Andrew E. Rosenberg, MD, and
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD Vikram Deshpande, MD
328 Pseudomyogenic Hemangioendothelioma 440 Gaucher Disease
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
334 Epithelioid Hemangioendothelioma
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
340 Angiosarcoma
G. Petur Nielsen, MD and Andrew E. Rosenberg, MD
xiii
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SECOND EDITION
NIELSEN | ROSENBERG
DESHPANDE • HORNICEK • KATTAPURAM • ROSENTHAL
This page intentionally left blank
SECTION 1
Bone Island/Osteopoikilosis 4
Osteoma 10
Osteoid Osteoma 16
Osteoblastoma 22
Bone Island/Osteopoikilosis
KEY FACTS
Benign Bone-Forming Tumors
4
Bone Island/Osteopoikilosis
ETIOLOGY/PATHOGENESIS CT Findings
• Small stellate medullary lesion with characteristics of
Neoplasm
cortical-type bone
• Cause of bone island is unknown
• Osteopoikilosis may be inherited in autosomal dominant Bone Scan
fashion • Can show some uptake of radionuclide
○ Associated with mutations and loss of function in LEMD3
located on 12q14 MACROSCOPIC
General Features
CLINICAL ISSUES
• Hard, solid, and tan-white; periphery blends into
Presentation surrounding trabeculae, which accounts for irregular
• Incidental radiographic finding and asymptomatic spiculated margins
○ Large bone islands may be painful
• Uncommon in children MICROSCOPIC
• Lesions in osteopoikilosis may undergo slow, progressive Histologic Features
enlargement or involution
• Consist of cortical-type bone containing haversian-like
Treatment canals
• Observation for small solitary lesions with classic • Predominately lamellar but may be focally woven
radiographic features • Osteoblasts lining surfaces are flat and quiescent
• Larger variants or cases in adolescents may require biopsy • Osteocytes are small and cytologically banal
to exclude more aggressive lesions, such as well- • Bone islands in osteopoikilosis are identical to sporadic,
differentiated osteosarcoma and sclerotic metastases in solitary bone islands
adults
DIFFERENTIAL DIAGNOSIS
Prognosis
• Excellent Well-Differentiated Osteosarcoma
• Malignant transformation does not occur • Infiltrative, composed of proliferating mildly atypical
spindle cells and woven bone
IMAGING Sclerotic Metastases
General Features • Usually in adults and contain histologically malignant cells
• Location
○ Most frequent sites are pelvis, ribs, and proximal femur DIAGNOSTIC CHECKLIST
○ In adults, incidence in pelvic bones (1.0%) and ribs (0.5%) Clinically Relevant Pathologic Features
○ Epiphyseal in tubular bones • Multiple lesions raise differential diagnosis of blastic
○ Osteopoikilosis is bilateral and symmetrical in metastases; multiple bone islands seen in osteopoikilosis
distribution and in metaphyseal and epiphyseal regions
of tubular bones Pathologic Interpretation Pearls
– Any bone may be affected, including tarsal and carpal • Lesion is intramedullary, cortical in type, and predominately
bones lamellar in architecture
• Size
○ Usually < 1 cm in diameter SELECTED REFERENCES
○ Infrequently, "giant" bone islands several cm in diameter 1. Korkmaz MF et al: Osteopoikilosis: report of a familial case and review of the
occur literature. Rheumatol Int. ePub, 2014
5
Bone Island/Osteopoikilosis
Benign Bone-Forming Tumors
6
Bone Island/Osteopoikilosis
7
Bone Island/Osteopoikilosis
Benign Bone-Forming Tumors
Osteopoikilosis Osteopoikilosis
(Left) AP radiograph
demonstrates osteopoikilosis
with multiple bone islands
involving the ends of the short
tubular bones. The small
lesions cluster at the ends of
the bones. (Right) AP
radiograph of the knees shows
the typical features of
osteopoikilosis. A large
number of small bone islands
are symmetrically distributed
in a primarily juxtaarticular
and metaphyseal distribution.
Despite their multiplicity, each
lesion individually has the
features typical of a bone
island.
Osteopoikilosis MR of Osteopoikilosis
(Left) Reformatted coronal CT
scan of the hip shows multiple
small bone islands that are
juxtaarticular and
metaphyseal in distribution.
Each lesion is individually
indistinguishable from a
solitary bone island. (Right)
T1-weighted MR of the knee in
a patient with osteopoikilosis
demonstrates that the bone
islands are small, rather
uniform in size, oval in shape,
and uniformly dense. Each
lesion is individually
indistinguishable from a
solitary bone island.
8
Bone Island/Osteopoikilosis
9
Osteoma
KEY FACTS
Benign Bone-Forming Tumors
TERMINOLOGY MACROSCOPIC
• Benign surface bone-forming tumor, usually composed of • Generally < 2 cm in diameter; round, tan-white, and hard
cortical-type bone • Resembles cortical bone with which it merges
CLINICAL ISSUES MICROSCOPIC
• Usually small and solitary • Consists mainly of lamellar bone admixed with some woven
• Commonly asymptomatic and incidental finding bone
• Most frequently develop in craniofacial skeleton • Bone has cortical-type architecture
• Appendicular tumors are very uncommon • Minority of osteomas composed of trabecular bone
• Multiple lesions raise possibility of Gardner syndrome • Lesional osteoblasts and osteocytes usually inconspicuous
• Asymptomatic lesions can be observed
DIAGNOSTIC CHECKLIST
• Symptomatic lesions can be conservatively excised
• Well-formed cortical bone and banal cytology distinguishes
IMAGING osteoma from osteosarcoma
• Small and uniformly radiodense • Intact cortex and absence of cartilage excludes
• Sharply marginated with well-formed periosteal reaction osteochondroma
• Oval to dome-shaped with broad attachment to cortical • Hypocellularity of lesion is evidence against myositis
surface ossificans
• Underlying cortex is not involved • Melorheostosis and osteoma are histologically similar
Radiograph of proximal lower leg shows a dense, well- Osteoma is composed of hard, dense, compact bone with a
defined ossific mass on the surface of the proximal fibula. broad attachment to the underlying cortex. A triangular-
There is thick, periosteal bone apposition proximal to the shaped zone of subperiosteal bone is present proximal and
mass that is triangular in shape. distal to the osteoma.
10
Osteoma
11
Osteoma
Benign Bone-Forming Tumors
12
Osteoma
13
Osteoma
Benign Bone-Forming Tumors
14
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Casa dell’Ancora.
La Casa di Cajo Vibio, scoperta in questi ultimi anni, è nella Regione
VII isola II N. 18: si distingue dalle altre per la solidità e la buona
conservazione delle mura. Vedine l’illustrazione nel numero d’Agosto
1868 del Giornale degli Scavi, nuova serie. Quella di Gavio Rufo è
vicina: porta il n. 16 ed è illustrata nello stesso giornale, numero di
settembre. Quella di Caprasio Primo è al N. 48. Di fronte è la
taberna di M. Nonio Campano. Vol. II, cap. XVII pag. 327.
Via delle Terme.
Terme pubbliche. Vol. II cap. XV pag. 207.
Casa del poeta. Vol. III cap. XX pag. 83.
Son presso de’ termopolii o venditorii di bevande calde. Vol. II, cap.
XVII.
Casa di Pansa edile, e secondo alcuni, di Paratus. Vol. III cap. XX
pag. 62.
Casa del maestro di musica, in cui v’è il musaico all’ingresso,
raffigurante il cane col motto Cave Canem. Id. pag. 85.
Fontana. Vol. II, cap. XV 226.
Forno e Mulini. Vol. II, cap. XVII.
Casa di Cajo Sallustio. Vol. III cap. XX.
Forno pubblico. Vol. II, cap. XVII pag. 307, passim.
Cisterna pubblica. Vol. II, cap. XV.
A destra è un vicolo che mette capo alla Via di Mercurio, e di fronte a
tal vicolo presentasi un atrio con all’intorno alcune camere, nelle
quali è installata la Scuola archeologica di Pompei.
Scheletri di una madre e di una figlia, di una matrona e d’un uomo.
Vol. I, cap. V.
Telonium o Dogana. Vol. I, cap. IV pag. 103.
Casa detta del Chirurgo.
Casa delle Vestali.
Termopolio. Vol. II.
Albergo. Idem.
Fortificazioni e porta d’Ercolano a tre archi colle traccie della
saracinesca nell’arcata di mezzo. Vol. I, cap. VII pag. 187.
Via delle Tombe. Vol. III, cap. XXII pag. 345.
A sinistra:
Tomba di M. Cerrinio. Id. Ibid. pag. 346. A schivare ripetizioni, da
questa pagina in avanti stanno, fino a pag. 368, le dichiarazioni delle
pur seguenti tombe.
Tomba di Vejo e suo emiciclo.
Monumento ed emiciclo di Mammia.
A destra:
Tomba delle ghirlande.
Gran nicchia per riposo dei visitatori.
Giardino delle colonne in musaico.
Albergo e scuderia.
A sinistra:
Il Pompejanum, o casa di Cicerone. Non soltanto in questo capitolo
XXII, ma anzi più largamente è trattato di essa anche nel Vol. I, cap.
III pag. 83.
Tomba di Scauro.
Tomba circolare.
A destra:
Tomba della porta di marmo.
A sinistra:
Mausoleo di Cajo Calvenzio Quieto. Anche nel Vol. I, cap. IV pag.
101.
Cippi della famiglia Istacidia.
Tomba di Nevoleja Tiche e di Munazio Fausto. Vedi anche nel Vol. I,
cap. VI pag. 101.
Triclinio funebre.
Tomba di Marco Allejo Lucio Libella padre e Marco Libella figlio.
Tomba di Cajo Labeone.
Tomba del fanciullo Velasio Grato.
Tomba del fanciullo Salvio.
Chiudesi la Via delle Tombe e la serie quindi di esse coi sepolcri
della famiglia Arria di Marco Arrio Diomede, di Marco Arrio
primogenito, di Arria l’ottava figlia di Marco, di un’altra Arria e di
quelli tutti della famiglia di Diomede.
Casa di campagna di Marco Arrio Diomede. Vedi anche Vol. I, cap. V
pag. 143, Vol. II, cap. XV e Vol. III, cap. XX pag. 87.
Visitata così tutta la parte della città che è esumata, alla estremità di
essa, al fianco opposto a quello delle Tombe che abbiamo appena
lasciato, al basso della Via di Stabia, dopo alcuni passi oltre gli
scavi, e a traverso de’ campi coltivati, che celano ancora parte della
città, si giunge all’Anfiteatro, del quale si son date in questa edizione
incise la fronte esterna nel titolo del secondo volume dell’opera e nel
corpo, la veduta interna. Vol. II cap. XIV.
Questa rapida corsa potrà durare quattro ore e, se appena il lettore
ha sentimento artistico, ne ritrarrà di certo da una prima visita il
desiderio di altre, le quali certo gli verranno rivelando nuove cose
degnissime di osservazione e di nota, e sarà allora, io spero, che gli
torneranno più accetti questi miei studi, nel compire i quali, non
fatica e stanchezza, ma diletto e conforto ho ricavato sempre contro
la cospirazione del silenzio e la viltà di politici avversari, le codarde
compiacenze di insipienti Eliasti e la stupidità degli Iloti onde
abbonda la nostra terra, Saturnia tellus, che mantien vivo l’appetito
del vecchio Nume divoratore de’ suoi figliuoli e che così ne
compensa le veglie sudate e le opere generose.
Non mi mancarono tuttavia i plausi de’ buoni e gli onesti ed onorevoli
incoraggimenti e poichè nel pigliar le mosse di questi miei studj, io
ne proclamavo auspice quel fior di senno e d’onestà che è il mio
carissimo Pietro Cominazzi; così piacemi chiuderli ancora nel suo
nome e il lettore non ascriva a mia vanità, ma al volere di
quell’egregio, se finisco qui riferendo i versi de’ quali egli per
quest’opera mi voleva onorato
A P. A. CURTI
Sonetto
Volume primo.
Volume secondo.
Volume terzo.
VOLUME PRIMO
Dedica Pag. V
Intendimenti dell’Opera VII
Introduzione 1