Memon O, Makhdoomi K R.
Dermatofibrosarcoma Protuberans presenting as Arteriovenous Malformation.
J Pak Assoc Derma Jan ;10(4):33-5.

Case Report Thirty-seven years old female presented to a dermatological clinic with a painful blue/ purplish discolored swelling on her right shoulder. The lesion was absent at birth and started 16 year ago. Initially it was small and painless but continued to increase in size and in last two years became symptomatic with pain and discomfort. She went to a general practitioner that referred her to a general surgeon who diagnosed it as AVM and advised conservative treatment. But as the pain got worse the general surgeon injected sclerosant into the lesion, which made the lesion pain free for a while, before deteriorating again. As the lesion increased in size she presented to a dermatologist who also thought the lesion was an AVM and referred her to our vascular clinic. The lesion was 7.5x4.0 cm protuberant with a purplish brown discoloration. It was indurated, non-compressible, non-pulsatile and tender to touch. Hand held Doppler was used to assess the vascularity, and the lesion was found to be highly vascular. A provisional diagnosis of AVM was made and a magnetic resonance imaging (MRI) of the right shoulder was performed to assess the depth of the AVM and invasion of the under lying structures. MRI revealed the lesion was indeed vascular with little invasion of the deep structures and it was thought to be an intradermal AVM.Because of the unusual history i.e. (absence of the any lesion at birth) ultrasound scan of the kidneys and thyroid were performed to rule out me tastasis from these organs. Because of the symptoms decision was made to excise the lesion. At the time of operation the lesion was thought to be a thrombosed AVM so a maximum of 0.5 cms clear margins were taken. The tissue was sent for histopathology, which revealed the lesion comprises of blood filled spaces with surrounding brown tissue on gross inspection. Microscopically it composed of intermingling of capillaries, fibroblasts, histiocytes and inflammatory cells. It showed focal collection of foamy histiocytes and occasional tuton giant cells, large blood filled spaces and extensive hemosiderin deposition. Focal nuclear pleomorphism and mitotic activity was seen. It was consistent with dermatofibrosarcoma protuberans.

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