PIGMENTED DERMATOFIBROSARCOMA PROTRUBERANCE (BEDNAR‘S TUMOR)–A RARE ENTITY.

Dr Shushruta Mohanty; Dr Lity Mohanty; Dr Pragyan Parimita Nayak; Dr Soudamini Mahapatra

Volume : VII, Issue : II, February - 2018

PIGMENTED DERMATOFIBROSARCOMA PROTRUBERANCE (BEDNAR‘S TUMOR)–A RARE ENTITY.

Dr Shushruta Mohanty, Dr Lity Mohanty, Dr Pragyan Parimita Nayak, Dr Soudamini Mahapatra

Abstract :

Bednar�s tumor is a rare skin neoplasm, considered to be a pigmented variant of der– matofiosarcoma protuberans. It accounts for approximately 1–5% of all cases of dermatofiosarcoma protuberans (DFSP). This tumor is of unexplained histogenesis but it may be related to remnants of emyonic mammary tissue or to various kinds of local trauma such as burns, vaccination scars and insect bites. Majority of lesions are located on the trunk, and the remainder are more or less equally distributed in the upper and the lower extremities and the head and neck and present as exophytic, multinodular neoplasms of the dermis or subcutaneous tissue . Microscopically the lesion is characterized by spindled cells arranged in a repetitive storiform pattern admixed with a small population of melanin–containing dendritic cells.. Bednar�s tumor is locally aggressive and recurrences are frequent, but metastases are rare. The most appropriate therapeutic procedure is Mohs‘ micrographic surgery. The case report presents a 35–year–old patient with this rare neoplasm in which the diagnosis was established through histopathologic examination and immunohistochemical study.