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

INTRODUCTION

Fibrosarcoma 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.

METHODS

A 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.

RESULTS

The age of presentation ranged from 2months to 75years (Median=17years). 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 1day to 8years (Median=15.5months).

CONCLUSION

Fibrosarcoma 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.