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.
Similar content being viewed by others
References
Bonnin JM, Rubinstein LJ (1984) Immunochemistry of central nervous system tumors. Its contribution to neurosurgical diagnosis. J Neurosurg 60:1121–1133
Dropcho EJ, Allen JC (1987) Primary intracranial rhabdomyosarcoma. Case report and review of the literature. J Neurooncol 5:139–150
Gaffney CC, Sloane JP, Bradley NJ, Bloom HJG (1985) Primitive neuroectodermal tumors of the cerebrum. J Neurooncol 3:23–33
Jalalah S, Kovacs K, Horvath E, Couldwell W, Weiss MH, Ezrin C (1987) Rhabdomyosarcoma in the region of the sella turcica. Acta Neurochir (Wien) 18:142–146
Matsukado Y, Yokota A, Marubayashi T (1975) Rhabdomyosarcoma of the brain. Case report. J Neurosurg 43:215–221
Olson JJ, Menezes AH, Godersky JC, Lobosky JM, Hart M (1985) Primary intracranial rhabdomyosarcoma. Neurosurgery 17:25–34
Rubinstein LJ (1972) Tumors of the central nervous system. Armed Forces Institute of Pathology, Washington, DC
Willis RA (1960) Pathology of tumors, 3rd edn. Butterworths, London
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Tomei, G., Grimoldi, N., Cappricci, E. et al. Primary intracranial rhabdomyosarcoma: report of two cases. Child's Nerv Syst 5, 246–249 (1989). https://doi.org/10.1007/BF00271028
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00271028