Intracranial fibrosarcoma treated with adjuvant radiation and temozolomide: Report of a case and review of all published cases

IntroductionFibrosarcoma is a rare brain tumour with 33 cases reported so far. However, there is no clear consensus about the nature of the disease and treatment as well as outcome.MethodsA MEDLINE search was carried out using MESH terms like intracranial fibrosarcoma, intraspinal fibrosarcoma, fibrosarcoma meninges and fibrosarcoma brain. A total of 22 case reports and series reporting a total of 33 cases were identified. We here also report a case treated in our institute with adjuvant radiation and concurrent and maintenance temozolomide.ResultsThe age of presentation ranged from 2 months to 75 years (Median = 17 years). The gender ratio was found to be M:F of 1.75–1. Treatment modalities were described for 17 cases. Surgery was part of treatment in all cases while radiation was a part of treatment in 59% of cases (n = 10) and chemotherapy in 29% cases (n = 5). Survival data were available only for 8 cases and ranged from 1 day to 8 years (Median = 15.5 months).ConclusionFibrosarcoma is a rare disease with dismal prognosis. Surgery remains the cornerstone of therapy. Radiation confers long term disease control and survival. Chemotherapy needs to be evaluated for these tumours to improve survival.