Neither the etiology nor the genetic pathways that lead to human meningosarcomas are clear; nevertheless, the finding of meningothelial elements within certain sarcomas has led some authors to hypothesize that the secondary sarcomatous changes in a preexisting meningioma might be considered as a meningosarcoma variant 1).
This difference is, in some instances, difficult to assess but still crucial given that the differences in both the histopathologic and genetic markers point to clinical entities which differ; specifically in terms of genomic instability which could play a role in the progression of some meningiomas 2).
First case published in the literature of meningeal sarcoma in a child in which intraoperative fluorescence with 5-ALA was used to achieve a complete resection 3).
Cummings et al. describe a boy with primary meningeal sarcoma who symptomatically presented at 10 months of age and was treated with primary resection. The patient had multifocal recurrence approximately 2 years later. Given the location and rapid progression of the disease, the boy was treated with gamma knife surgery. He had a complete radiographic response 3 years posttreatment. He attends school full time and enjoys good quality of life. Based on local control and response to radiosurgery, the authors suggest that multifocal meningeal sarcomas not amenable to resection can be effectively managed with stereotactic radiosurgery 4).
Hong et al. present the case of a 25-year-old woman in the third trimester of pregnancy with a large intracranial tumor destructing the parietal calvaria and invasion of soft tissues. Histological examination revealed primary meningeal sarcoma with leiomyoblastic differentiation. A gross macroscopical resection of the tumor with removal of the infiltrated parietal calvaria was performed after delivery of a healthy baby through caesarean section. A mass on the head rapidly enlarging during pregnancy should be considered for a malignancy of intracranial origin. Early radiological exams as well as tumor resection followed by staging and multimodality treatment should be urgently performed 5).