Primary intracranial rhabdomyosarcoma: report of two cases

Giustino Tomei, Nadia Grimoldi, Enrico Cappricci, Erik P. Sganzerla, Sergio M. Gaini, Roberto Villani, Battistina Masini

Research output: Contribution to journalArticlepeer-review

Abstract

Primary intracranial rhabdomyosarcoma (RMS) is a rare tumor in infancy and childhood that is found in various locations in the central nervous system. The clinical course worsens rapidly, and the final outcome is poor, with a median survival time of 8-10 months. Invasion of the meninges, spontaneous intratumoral bleeding, spinal leptomeningeal CSF spreading of tumor cells, and early recurrence of the mass are the distinctive features of RMS. Diagnosis of RMS may be missed: immunohistochemical staining using specific markers (myoglobin, myosin, desmin, vimentin, enolase), along with ultrastructural studies, provide the basis for making the final diagnosis. Treatment of RMS includes surgical excision, craniospinal radiation therapy, and chemiotherapy. We report two cases of primary RMS in the CNS located in the posterior fossa and frontotemporal area. Both children underwent total surgical removal of the mass. Early recurrence of the tumor mass was noticed in both patients 2 months after surgery. Both children died shortly thereafter.

Original languageEnglish
Pages (from-to)246-249
Number of pages4
JournalChild's Nervous System
Volume5
Issue number4
DOIs
Publication statusPublished - Aug 1989

Keywords

  • Immunochemistry
  • Intracranial rhabdomyosarcoma
  • Intratumoral hemorrhage

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Fingerprint Dive into the research topics of 'Primary intracranial rhabdomyosarcoma: report of two cases'. Together they form a unique fingerprint.

Cite this