An unusual case of turban tumor syndrome treated with total scalp excision and advancement flap and skin graft reconstruction.

We report an unusual case of aggressive turban tumor syndrome in a 38-year-old woman with nodules covering her scalp and involving her face, neck, chest, and back. To address concerns regarding hygiene of the scalp and cosmetic disfigurement, she underwent total scalp excision with advancement flap and skin graft reconstruction. Histologic examination of the scalp tumors revealed a predominance of spiradenomas, along with cylindromas and trichoepitheliomas, and tumors containing elements of all 3 of these adnexal neoplasms. We review the literature regarding turban tumor syndrome including the genetic basis for this condition, clinical features, pathology, and treatment.