Dermatofibrosarcoma protuberans of scalp with cervical lymph node metastasis

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, slow growing and locally aggressive tumor of the skin with a high rate of recurrence even after supposedly wide excision. The reports of regional lymph node metastasis and distant metastasis are very rare. Because of the extreme rarity of these cases with metastasis, the experience with management of such patients is very limited. A case of recurrent DFSP of scalp, with metastasis to the regional lymph nodes, in a 17-year-old boy is reported here. This is the second case of DFSP involving scalp and 16th case of DFSP of all sites metastasizing to the regional lymph nodes reported in literature. The patient was treated with wide excision of the lesion and ipsilateral radical neck dissection (including excision of overlying involved skin).