Sarcomas in the Central Nervous System of Children

Sarcomas in the Central Nervous System of Children Fernando Rueda-Franco, MD, Amargura 46-1 – San Angel, Mexico City, DF, 01000 (Mexico) Introduction The neurosurgeon encounters sarcomatous lesions either as primary diseases of the central nervous system or its coverings or as lesions originating elsewhere, secondarily involving the nervous system often after having been diagnosed and treated at their primary site. The former, usually manifested as rapidly progressive space-occupying lesions, fall obviously in his domain but he is also frequently sought for palliative treatment of neurologic manifestations of extraneural neoplasms. Moreover, extraneu-ral tumors not infrequently manifest themselves initially with neurologic signs and symptoms. Patients with these infrequent and highly aggressive lesions usually undergo a conventional neurosurgical approach and it is the pathological examination that reveals the unsuspected nature of the tumor and determines further therapeutic decisions. Thus, a collective review of both primary and secondary sarcomatous lesions involving the nervous system, although nosologically unorthodox, claims the practical advantage of grouping together diverse lesions that share common problems for the neurosurgeon. Intracranial and intraspinal sarcomas constitute an ill-defined group in terms both of taxonomy and nomenclature. Classifications have varied according to the personal viewpoint of different authors [1-5] and the boundaries of the group are as yet not completely established. Lesions such as the so-called monstruocellular sarcoma [1] are considered as either a form of glioblastoma or as a sarcoma of the blood vessel origin. Reticulum cell sarcomas/microgliomas are in the process of being incorporated into the lymphomas [6] but this view is not unanimous. The purpose of this paper is to report on the analysis of a group of 26 patients with intracranial or intraspinal sarcomas attended over a 10-year period at the Instituto Nacional de Pediatría-DIF in Mexico City. We included in this series all patients with sarcomatous lesions identified either at surgery or at autopsy as involving the central nervous system. Lesions considered lymphomatous in nature were excluded. Case Material Frequency and Distribution One of our patients had both intraspinal and intracranial involvement giving a total of 27 lesions; 15 of these were intracranial and 12 were intraspinal. Sarcomas thus represent 5.5% of our series of 270 intracranial tumors. This figure is higher than Matson’s [7] 8 sarcomas