Turban Tumor Syndrome: In Search of a Gold Standard – A Case Report

Turban tumor syndrome (TTS) is a rare condition characterized by predisposition to developing multiple adnexal tumors. It is the rare autosomal dominant genodermatosis that is associated with the mutation of the CYLD suppressor gene. We herein report rare TTS in an adult who suffered from numerous episodic ulcerations of the scalp tumors and frequent bleeding following a minor trauma. The plain radiograms of the skull excluded bone marrow infiltration, and no regional lymphadenopathy was noted. The patient underwent total scalp excision and split skin grafting under general anesthesia in a single-stage operation. Recovery was uneventful. No recurrence was observed during the 2-year follow-up. Due to a high recurrence rate and risk of neoplastic transformation, surgery should be considered as the gold standard.

Brooke-Spiegler Syndrome with Multiple Scalp Cylindromas and Bilateral Parotid Gland Adenomas

A 62-year-old female presented with numerous soft tissue lesions of her scalp and bilateral preauricular region. Several of these have been biopsied or removed with a diagnosis of cylindromas. Cylindromas are benign tumors with a differentiation towards apocrine sweat glands that increase in number and size throughout life. Multiple scalp cylindromas may coalesce and cover the entire scalp, resulting in the “turban tumor.” These are often associated with the autosomal dominant Brooke-Spiegler syndrome with coexistent facial trichoepitheliomas and spiradenomas. There is a very rare association between cylindromas and basal cell adenoma and adenocarcinoma of the parotid gland, with only 17 reported cases. Ours is the first CT demonstration of both the scalp and parotid gland findings in this uncommon situation.