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

الملخص EN

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 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).

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.