Primary intracranial Rhabdomyosarcoma: Case report and review of the literature

Edward J. Dropcho, Jeffrey C. Allen

Research output: Contribution to journalArticle

28 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)139-150
Number of pages12
JournalJournal of Neuro-Oncology
Volume5
Issue number2
DOIs
StatePublished - Jun 1 1987
Externally publishedYes

Keywords

  • brain neoplasms
  • cerebral hemorrhage
  • meningeal neoplasms
  • rhabdomyosarcoma

ASJC Scopus subject areas

  • Oncology
  • Neurology
  • Clinical Neurology
  • Cancer Research

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