Primary meningeal sarcoma in children: a survey from the French Society of Pediatric Oncology (SFCE)

Objectives: To describe the outcome of primary meningeal sarcoma (PMS) in a pediatric population. Methods: We conducted a retrospective study on patients harbouring a PMS based on the French registry of paediatric tumours. Questionnaires were sent to all French paediatric oncologists. Data on patient characteristics and treatments were collected. Pathology and imaging were centrally reviewed. Results: Between August 1989 and May 2010, 12 patients from 6 French centres, aged 3 months to 14.5 years (mean: 3.3 years) were treated for a PMS. Mean follow-up was 12 years (range: 3 months to 24 years). Tumour locations were: frontal (3), parieto-occipital (2), parietal (1), temporal (1), occipital (1), thalamic (1), pontocerebellar angle (1), cerebellar tentorium (1), ambient cistern (1). No metastasis was observed. The first-line treatment was surgery in 10 cases, chemotherapy in 2. Resection was total in 6 cases, partial in 6. Central pathology review concluded to: high-grade undifferentiated sarcoma (8), chondrosarcoma (2), fibrosarcoma (1), myxoid desmoplastic tumour (1). Seven tumours were smaller than 5 cm in size. Eleven patients received 2 to 10 courses of chemotherapy (median: 5) but no response was observed. Four out of 6 patients for whom a total resection was performed were still alive at time of study, compared to 2 when only partial resection was possible. Four out of 6 patients who received radiotherapy are still alive, compared to 1 out of 4 patients without radiotherapy. The 5-year Event-Free Survival (EFS) and Overall Survival (OS) rates were 50%. The median EFS in case of total resection was 39 months versus 16 months in case of partial resection. Neither correlation was found between tumour size and location or between age at diagnosis and EFS/OS. Conclusions: We report the first multicentre case series describing clinical, radiological presentation and outcome of PMS in children. In this short series of very rare tumours, age and tumour size did not seem to be prognostic factors. Total resection and radiotherapy seem to be essential. The role of chemotherapy remains unclear.