Article

Pediatric primary pilocytic astrocytoma of the cerebellopontine angle: a case report.

Department of Neurosurgery, Lariboisiere University Hospital, Paris, France.
Child s Nervous System (impact factor: 1.54). 09/2008; 25(2):247-51. DOI:10.1007/s00381-008-0690-9 pp.247-51
Source: PubMed

ABSTRACT We describe a rare case of pediatric pilocytic astrocytoma presented as a right cerebellopontine angle (CPA) mass, completely separated from the brain stem and arising from the proximal VIII nerve portion.
A 12-year-old boy, with no evidence of neurofibromatosis type 2, presented with progressive hearing loss at the right ear and headache. An initial enhanced magnetic resonance examination suggested the diagnosis of schwannoma. The tumor was resected by a suboccipital retrosigmoid approach.
Our case seems to be the first report of a primary pediatric CPA pylocitic astrocytoma arising from the VIII nerve complex and presenting internal auditory canal enlargement. It represents the third reported case of a primary CPA pilocytic astrocytoma (the second pediatric case with the first arising from V nerve) and the eighth report of primary CPA glioma, overall. We discuss the clinical, neuroradiological, and intraoperative findings, and we review the different hypothesis about the origin of these rare tumors.

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    Aline Paixão Becker, Ricardo Santos de Oliveira, Fabiano Pinto Saggioro, Luciano Neder, Leila Maria Cardão Chimelli, Hélio Rubens Machado
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    ABSTRACT: This study described a 23-year experience in the treatment of children with pilocytic astrocytomas (piloA) with the aim of identifying putative clinical, histopathological, and/or immunohistochemical features that could be related to the outcome of these patients. Clinical data of 31 patients under 18 years of age with piloA were obtained from 1984 to 2006. The mean age at the time of surgery was 7.8 +/- 4.2 years (1 to 17 years), and the mean follow-up was 5.7 +/- 5.4 years (1 to 20 years). The most common site of tumor formation was the cerebellum (17), followed by brainstem (4), optic chiasmatic hypothalamic region (4), cerebral hemisphere (3), cervical spinal cord (2), and optic nerve (1). Gross total resection (GTR) was achieved in 23 (74.1%), mainly in those with tumors located in the cerebellum and cerebral hemispheres (P = 0.02). The global mortality rate was 6.4%. Nine patients were reoperated. Rosenthal fibers, eosinophilic granular bodies, microvascular proliferation, and lymphocytic infiltration were observed in most cases. The mean Ki-67LI was 4.4 +/- 4.5%. In all cases, Gal-3 expression in tumor cells was observed with variable staining pattern. Aside from GTR, no other clinical, histopathological, or immunohistochemical features were found to be related to the prognosis. We postulate that strict follow-up is recommended if piloA is associated with high mitotic activity/Ki67-LI, or if GTR cannot be achieved at surgery. Tumor recurrence or progression of the residual lesion should be strictly observed. In some aspects, childhood piloA remains an enigmatic tumor.
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Keywords

clinical
 
different hypothesis
 
eighth report
 
first report
 
initial
 
internal auditory canal enlargement
 
intraoperative findings
 
neurofibromatosis type 2
 
pediatric pilocytic astrocytoma
 
primary pediatric CPA pylocitic astrocytoma
 
progressive hearing loss
 
proximal VIII nerve portion
 
rare case
 
rare tumors
 
second pediatric case