Primary intracranial spindle cell sarcoma with rhabdomyosarcoma-like features in pediatric patients: cases series 2020-2021

Abstract

Primary intracranial sarcoma, is a non-meningothelial mesenchymal tumor of uncertain differentiation, although similar to its soft tissue counterparts, it is regularly found in the CNS. They are generally reported as metastases from
extracranial sarcomas, with presentation in the central nervous system being very rare. Likewise, the radiological representation of this tumor is limited by the few cases reported in the international literature, especially in the
pediatric population. A series of 4 cases of pediatric patients under 8 years of age from Lima - Peru is presented, who present sudden onset intense headache associated with continuous vomiting, in some with signs of focalization or
convulsive symptoms. The magnetic resonance studies mostly showed an extensive expansive tumor lesion with a solid appearance with thickening and dural enhancement, located in the supratentorial region in 75% of the cases and
peripheral distribution. These lesions presented restriction to the diffusion study and magnetic susceptibility artifact of peripheral and central distribution, suggestive of nonrecent bleeding, with intraparenchymal extension and with
signs of hydrocephalus. Because it is an extremely rare pathology with few publications worldwide, it is important to present these cases to expand the radiological description and reduce the differential diagnoses, to determine their
surgical management and subsequent controls due to the high rate.