Rhabdoid choroid plexus carcinoma: a rare histological type.

Abstract:

:Primary central nervous system atypical teratoid/rhabdoid tumors mostly occur during early childhood and are almost invariably fatal. These tumors show similar histological and radiological features to primitive neuroectodermal tumor, medulloblastoma and choroid plexus carcinoma, but present different biological behaviors. We present the case of an 18 year-old man who presented headache, vomiting and ataxia. CT-scan and MRI revealed a posterior fossa tumor. A gross total resection was performed. An intraoperative study showed papillary-like tumors with large cells and mitotic features. Histological examination showed two different main growth patterns: solid sheets of undifferentiated polygonal cells with papillary features and rhabdoid cells. Immunohistochemically, these rhabdoid cells were positive for vimentin, epithelial membrane antigen, smooth-muscle actin, cytokeratin, S-100 protein, and glial fibrillary acidic protein. Electro-microscopically, the typical rhabdoid cells contained whorled bundles of intermediate filaments in their cytoplasm. A rhabdoid tumor is a clinical-pathological entity and emphasizes the necessity to distinguish this unique tumor from other pediatric central nervous system neoplasms. Cytopathological features, immunohistochemistry and electro-microscopy differential diagnoses are discussed.

journal_name

Arq Neuropsiquiatr

authors

Tena-Suck ML,Gómez-Amador JL,Ortiz-Plata A,Salina-Lara C,Rembao-Bojórquez D,Vega-Orozco R

doi

10.1590/s0004-282x2007000400032

subject

Has Abstract

pub_date

2007-09-01 00:00:00

pages

705-9

issue

3A

eissn

0004-282X

issn

1678-4227

pii

S0004-282X2007000400032

journal_volume

65

pub_type

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