Pilomatrixoma of the head and neck.

Pilomatrixoma is a benign skin appendage tumor that commonly occurs as a solitary lesion and is not usually hereditary. There is evidence to suggest that patients with a family history of multiple pilomatrixomas have a high probability of autosomal dominant disorders such as myotonic dystrophy, Gardner syndrome, and Rubinstein-Taybi syndrome. In January 2004, a case was reported of an 8-year-old girl with 2 progressively enlarged facial masses. One of them was excised and diagnosed as pilomatrixoma. Coincidently, her 13-year-old sister had the same type of tumor when she was 4 years old. We report this unusual case and review the literature. Pilomatrixoma has not been widely reported in the head and neck surgery literature. This benign tumor may be misdiagnosed as a carcinoma, resulting in unnecessary aggressive therapy. Otolaryngologists should therefore note the clinical and pathologic characteristics of these symptoms.