Open AccessTurban Tumor Syndrome: In Search of a Gold Standard – A Case Report
Author(s) -
Jakub Bargiel,
Grażyna Wyszyńska-Pawelec,
Michał Gontarz,
Krzysztof Gąsiorowski,
Tomasz Marecik,
Paweł Szczurowski,
Jan Zapała
Publication year - 2021
Publication title -
skin appendage disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.773
H-Index - 13
eISSN - 2296-9195
pISSN - 2296-9160
DOI - 10.1159/000514855
Subject(s) - medicine , scalp , skull , surgery , genodermatosis , malignant transformation , gold standard (test) , dermatology , radiology , pathology , biochemistry , chemistry , gene
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.