Fibrosarcomatous dermatofibrosarcoma protuberans of the scalp, extending to the brain – A rare case report

Abstract

This case report highlights a rare instance of fibrosarcomatous dermatofibrosarcoma protuberans of the scalp with extension to the brain. Dermatofibrosarcoma protuberans is a slow-growing, locally aggressive soft tissue tumor. Fibrosarcomatous dermatofibrosarcoma protuberans is a rare and more aggressive variant of dermatofibrosarcoma protuberans. Fibrosarcomatous transformation in dermatofibrosarcoma protuberans is observed in only 5 % of cases. Although the recurrence rate is higher, metastasis and local extension are rarely seen, and intracranial extension is also uncommon. Clinically, fibrosarcomatous dermatofibrosarcoma protuberans is indistinguishable from classical dermatofibrosarcoma protuberans; hence, histopathological examination remains the cornerstone for definitive diagnosis. Histopathological examination reveals that areas of classical dermatofibrosarcoma protuberans show tumor cells arranged in a monotonous storiform pattern with a lack of circumscription, while areas with fibrosarcomatous transformation exhibit spindle cells arranged in a herringbone pattern, which are negative for CD34 on immunohistochemistry.