Surgical Management Of A Dermatofibrosarcoma Protuberance Of The Face

摘要

Background: Dermatofibrosarcoma Protuberance(DFSP) is a rare cutaneous tumor of unknown etiology. The lesion frequently develops on the extremities (mostly the lower legs) trunk, and rarely above neck. It is usually asymptomatic, although pruritus and tenderness are not uncommon. DFSP is a cutaneous malignancy that arises from the dermis and invades deeper subcutaneous tissue (eg, fat, fascia, muscle, bone).Two cases of dermatofibroma of the face presented to us of which is a pigmented variety also called bednar [6] . WE report these cases as these tumors are rare and though an entity known for about 9 decades still much is research is to be done regarding the management.Materials & Methods : Two cases of Dermatofibrosarcoma Protuberance of face of which one was pigmented variety, presented to Plastic Surgery OPD between July 2003 to December 2005.These cases were managed by wide excision and reconstruction by local rotational flaps to achieve cosmetically good results. Introduction Primary mesenchymal tumours with localization outside the skeleton, parenchymatous organs or hollow viscera are generally referred to as soft tissue sarcomas. These are slow growing tumors Dermatofibrosarcoma protuberans (DFSP) is a relatively unusual, highly invasive locally aggressive cutaneous tumour of intermediate malignancy.Usually this is reported to occur in limbs and trunks, incidence on face is very rare. Moreover the pigmented variety is still a rarer entity. [7]The age of patients vary between second to forth decade, but may present as late as 80 years. These tumors are locally invasive, but recurrence rate is found to be very high.Surgery forms the mainstay of management, though radiotherapy has got some role incases of involved margins. Chemotherapy has no role in management. History Two female patients with exfoliative growth at face presented to us between July 2003 to December 2005.On histopatholgy these lesions came out to be pigmented dermatofibrosarcoma protuberance a locally invasive malignant tumor which was cosmetically unacceptable in these patients. CASE 1 Mrs ABC a 60 years old female presented to us with a huge mass of size of 13 cm. x 9 cm arising from the bridge of nose and inner canthus of the left eye. It was covering both the eyes as a curtain. It was pink colored with areas of necrosis. No cervical lymph nodes were palpable. Histopathology revealed pigmented dermatofibrosarcoma protuberance. [Photo 1]