Primary intracranial rhabdomyosarcoma: Case report and review of the literature Journal Article


Authors: Dropcho, E. J.; Allen, J. C.
Article Title: Primary intracranial rhabdomyosarcoma: Case report and review of the literature
Abstract: Invasion of the meninges is a relatively common complication of head and neck rhabdomyosarcoma (RMS), while RMS arising primarily within the brain or meninges is rare. We report the case of an 11-year old child with a primary "primitive" frontal lobe tumor, subsequent leptomeningeal spread and fatal intratumoral hemorrhage; the diagnosis of RMS was discovered only at postmortem examination. The literature contains a total of 34 reported cases of primary intracranial RMS. This tumor has been observed to arise in a variety of central nervous system (CNS) locations in patients of all ages, but most commonly within the posterior fossa of children. Leptomeningeal dissemination and spontaneous intratumoral hemorrhage are important clinical features. Postoperative chemotherapy and craniospinal radiation may improve the anticipated poor prognosis of patients treated with surgery and radiation alone. The diagnosis of RMS may be missed unless electron microscopic and specific immunohistochemical studies are applied to "undifferentiated" or "primitive" CNS tumors. © 1987 Martinus Nijhoff Publishers.
Keywords: child; school child; case report; chemotherapy; brain neoplasms; carboplatin; radiotherapy; tomography, x-ray computed; dexamethasone; histology; central nervous system; image enhancement; meningeal neoplasms; bone; craniotomy; seizure; meningioma; rhabdomyosarcoma; autopsy; organoplatinum compounds; therapy; neuropathology; cerebral hemorrhage; nervous system; human; female; support, non-u.s. gov't
Journal Title: Journal of Neuro-Oncology
Volume: 5
Issue: 2
ISSN: 0167-594X
Publisher: Springer  
Date Published: 1987-06-01
Start Page: 139
End Page: 150
Language: English
DOI: 10.1007/bf02571302
PUBMED: 3312510
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 5 February 2021 -- Source: Scopus
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