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

Abdullah Almasoud, Emmanuelle Schmitt, Jacques Grill, Didier Frappaz, Jean-Claude Gentet, Sandrine Pall-Kondollf, Marie-Amelyne Le Rouzic, Julie Valduga


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.


meningeal sarcoma; child, surgery; chemotherapy; radiotherapy; outcome

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Dolecek TA, Propp JM, Stroup NE, et al. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2005-2009. Neuro Oncol 2012; 14:1-49.

Al-Gahtany M, Shroft M, Bouffet E, et al. Primary central nervous system sarcomas in children: clinical, radiological, and pathological features. Childs Nerv Syst 2003;19:808–817.

Hsü Yk. Primary intracranial sarcomas. Arch Neurol Psychiat 1940; 43:901-924.

Onofrio BM, Kernohan JW, Uihlein A. Primary meningeal sarcomatosis. A review of the literature and report of 12 cases. Cancer 1962; 15:1197–1208.

Budka H, Pilz P, Guseo A. Primary leptomeningeal sarcomatosis. Clinico-pathological report of six cases. J Neurol 1975; 211:77–93.

Kishikawa T, Numaguchi Y, Fukui M, et al. Primary intracranial sarcomas: radiological diagnosis with emphasis on arteriography. Neuroradiology 1981; 21:25–31.

Davidson GS, Hope JK. Meningeal tumors of childhood. Cancer 1989; 63:1205-1210.

Hope JK, Armstrong DA, Babyn PS, et al. Primary meningeal tumors in children: correlation of clinical and CT findings with histologic type and prognosis. Am J Neuroradiol 1992; 13:1353–1364.

Büttner A, Pfluger T, Weis S. Primary meningeal sarcomas in two children. J Neurooncol 2001; 52:181–188.

Cummings M, Chowdhry V, Shah H, et al. Recurrent meningeal sarcoma successfully treated with stereotactic radiosurgery. J Neurosurg Pediatr 2012; 10:434-438.

Singla N, Kapoor A, Chatterjee D. Undifferentiated meningeal sarcoma of childhood presenting as hard mass adhered to major intracranial vessels. Childs Nerv Syst 2016; 32:771-773.

Paulus W, Slowik F, Jellinger K. Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 1991; 18:395-402.

Wang XQ, Jiang CC, Zhao L, et al. Clinical features and treatment of World Health Organization Grade II and III meningiomas in childhood: report of 23 cases. J Neurosurg Pediatrics 2012; 10:423–433.

Benesch M, von Bueren AO, Dantonello T, et al. Primary intracranial soft tissue sarcoma in children and adolescents: a cooperative analysis of the European CWS and HIT study groups. J Neurooncol 2013; 111:337–345.

Maher OM, Khatua S, Mukherjee D, et al. Primary intracranial soft tissue sarcomas in children, adolescents, and young adults: single institution experience and review of the literature. J Neurooncol 2016; 127:155–163.

Haddad GF, Al-Mefty O. Meningeal sarcoma. In: Kaye AH, Laws ERJ (eds). Brain tumors. An encyclopedic approach. Churchill Livingstone, Edinburgh; 1995. p 713-721.

Rubinstein L, Russell D. Pathology of Tumours of the Nervous System. 5th ed. London: Edward Arnold; 1989. p. 507–532.

K Ravindranath, MC Vasudevan, A Pande, et al. Management of pediatric intracranial meningiomas: an analysis of 31 cases and review of literature. Childs Nerv Syst 2013; 29:573–582.

Dziuk TW, Woo S, Butler EB, et al. Malignant meningioma: an indication for initial aggressive surgery and adjuvant radiotherapy. J Neurooncol 1998; 37:177-188.

Copyright (c) 2018 Abdullah Almasoud, Emmanuelle Schmitt, Jacques Grill, Didier Frappaz, Jean-Claude Gentet, Sandrine Pall-Kondollf, Marie-Amelyne Le Rouzic, Julie Valduga

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