IJSR International Journal of Scientific Research 2277 - 8179 Indian Society for Health and Advanced Research ijsr-7-2-14279 Original Research Paper PIGMENTED DERMATOFIBROSARCOMA PROTRUBERANCE (BEDNAR‘S TUMOR)–A RARE ENTITY. Shushruta Mohanty Dr. Dr Pragyan Parimita Nayak Dr. February 2018 7 2 01 02 ABSTRACT

 

Bednar’s tumor is a rare skin neoplasm, considered to be a pigmented variant of der– matofibrosarcoma protuberans. It accounts for approximately 1–5% of all cases of dermatofibrosarcoma protuberans (DFSP). This tumor is of unexplained histogenesis but it may be related to remnants of embryonic 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.