Introduction:

Pilocystic astrocytoma is a brain tumor that occurs more in children and young
adults. They usually araise from the cerebelum,near the brainstem in the
hypothalamic region. These tumors are usually slow growing and benign.

Definition of Pilocytic astrocytoma

Pilocytic astrocytoma, the most common childhood brain tumor, is typically associated with
mitogen-activated protein kinase (MAPK) pathway alterations. Surgically inaccessible midline
tumors are therapeutically challenging, showing sustained tendency for progression and often
becoming a chronic disease with substantial morbidities.

accounting for ~20% of all pediatric brain tumors. Tumor locations in our cohort reflect the fact
that pilocytic astrocytomas occur throughout the CNS, with about half arising outside the
cerebellum. Extra-cerebellar tumors are often surgically inaccessible, leading to a chronic
disease with multiple recurrences, visual and neurological impairment, and/or side-effects of
therapy.

Histopatholgy of Pilocytic astrocy toma

Health Organization (WHO) as grade I neoplasms, which typically have an excellent prognosis.
In this regard, tumors amenable to gross total resection (GTR) are considered " cured”, with low
risk of tumor recurrence following resc-tion. However, PAs arising in the optic
pathway,brainstem, and diencephalon are not usually amenable to GTR. Surgery for tumors in
these regions is typically sub-total in order to avoid complications, such as visual Weld loss,
third nerve palsy, endocrine deWcits, hypothalamic obesity, and death. In these situations where
GTR is not possible, there is signiWcant variability in the behavior of PAs. The diYculty in
predicting clinical outcome for such patients underscores the need for prog-nostic genetic or
histopathologic biomarkers. To identify histopathologic features associated with tumor
recurrence, we performed detailed clinical, routine pathologic, and immunohistochemical
analyses on a series of PAs from 107 patients where the clinical course was known. To our
knowledge, this is one of the largest series examining the clinical signiWcance of these variables
in PAs. In this report, we speciWcally found that the presence of necrosis, oligodendroglioma-
like cytology, vascular hya- linization, and calciWcation were each associated with reduced
event-free survival (EFS). In addition, the increase of CD68+ cells showed a trend towards
worse EFS. This intriguing Wnding suggests that elements of the tumor envi -ronment, such as
actively proliferating monocytic or microglial cells may contribute to tumor growth in this oth -
erwise benign neoplasm. In addition to standard histologic review, the maximal number of
mitoses per ten consecutive high powered fields (HPF) was determined on a careful search of all
available slides, and the MIB-1 (Ki-67) proliferative labeling index (LI) was calculated for each
specimen. The latter was calculated as a maximal labeling index (percentage) by counting the
fraction of immunopositive nuclei among 500–1000 non-endothelial cells (1–3 HPF) within “hot
spots” identified on low-magnification scanning. High proliferative indices were defined as >1
mitosis/10 HPF or >0.5% MIB-1 LI . Slides were also specifically examined for the presence or
absence of biphasic pattern, microcyst formation, Rosenthal fibers, eosinophilic granular bodies,
oligodendroglioma-like cytology, vascular hyalinization, chronic inflammation,
microcalcifications, hemorrhage, microvascular proliferation (e.g., glomeruloid vessels with
multiple lumens and endothelial prominence), necrosis, leptomeningeal invasion, and
perivascular pseudorosettes such as those seen more extensively in pilomyxoid astrocytomas.

Who grading?

Pilocytic astrocytomas WHO grade I (PA I) and diffuse astrocytomas WHO grade II (A II)
exhibit characteristic morphological features allowing to distinguish these tumors in most
instances .While, PA I usually are of low cellularity and are characterized by elongated or
bipolar tumor cells accompanied by Rosenthal fibers and a typical vascular pattern with
hyalinized and sometimes glomeruloid vessels, A II mostly present with uniform and
moderately dense astrocytic cells in a loosely structured often microcystic stroma with
inconspicuous vessels. However, morphological variety and limited material at examination
could result in major difficulties to distinguish PA I from A II. For example, rare variants of PA
I such as the so-called diffuse pilocytic astrocytoma of the cerebellum mimicking the histology
of A II are difficult to discern on morphological grounds only. Another frequent obstacle for
straightforward diagnosis is small sample size due to tumor localization in sensitive areas or
due to techniques such as stereotactic biopsy. In such cases, additional tumor specific markers
besides histological features would greatly assist diagnosis.

Mutations in the gene encoding human cytosolic NADP+ dependent isocitrate dehydrogenase
(IDH1) recently have been reported to be very frequent, approaching 70–80% in A II, while
being rare in PA I. On the other hand, novel findings established a DNA copy number gain at
chromosomal band 7q34 including the human v-raf murine sarcoma viral oncogene homolog
B1 gene (BRAF) as a typical lesion for PA I, seen in approximately 70%, but much less
frequent in A II Recent studies have found that copy number gain at 7q34 is due to a tandem
duplication resulting in fusion of BRAF and KIAA1549 genes, which lacks the auto-inhibitory
domain of wild type BRAF . This event results in activation of the ERK/MAPK pathway and
promotes G2/M transition in the cell cycle .

Gross features

In 1953, a 9 y.o. boy was examined at our institution because of headaches, nausea, vomiting
and ataxia. A pneumoventriculography revealed a subtentorial space occupying mass, which
was surgically explored. During surgery a large cystic cavity was disclosed in the midline, with
a solid mural nodule clinging to the lateral wall. Ac -cording to the surgeon's opinion, the solid
nodule was entirely re-moved. The cystic wall had the aspect of normal cerebellar cortexand
was not removed. Surgical samples were processed for light mi -croscopy. Bouin ®xative was
used immediatly after surgical removal.4mm para½n embedded sections were stained with
haematein-phloxin-saphran, Masson's trichrome and PAS. Immunohisto-chemical test (anti-
GFAP) was performed 45 years later. Histo-logical examination was characterized by two
patterns: a loosely knittissue and a more compact one. The former was composed of stellate

astrocytic cells, GFAP-positive, in areas ®lled with mucinous back -ground; the latter contained
piloid elements with Rosenthal ®bersand brightly eosinophilic granular bodies. Nuclear
polymorphism was common,

concolusion

pilocystic astrocytomas are the most common pediatric brain tumors in our population.

Complete resection is the best treatment option but some tumors are aggrassive and recurrence.