Dermal cylindroma of the scalp (turban tumour) and subjacent calvarian defects.
This case report describes the diagnosis and therapy of a patient with a 40-year history of multiple potato-like tumours growing in the head and neck region. The tumour proved to be a cylindroma, associated with calvarian defects. Further facial tumours were diagnosed as trichoepitheliomas. This association of findings was pathognomonic for Brooke-Spiegler's syndrome. Complete work-up of the resection specimen excluded any malignant transformation of the tumour. A long lasting history of cylindroma and evidence for bone destruction, not associated with a malignant transformation, is extremely rare in Brooke-Spiegler's syndrome.
Friedrich
Reinhard E
RE
Department of Oral and Maxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany. rfriedrich@uke.uni-hamburg.de
D000368:Aged; D000792:Angiography / Q000379:methods; D001706:Biopsy; D003528:Carcinoma, Adenoid Cystic / Q000175:diagnosis* / Q000473:pathology*; D002339:Carotid Arteries / Q000473:pathology; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis* / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D012535:Scalp / Q000473:pathology*; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000473:pathology*; D013577:Syndrome; D016896:Treatment Outcome