Abstract / Summary
Cutaneous cylindroma is an uncommon adnexal neoplasm of presumed follicular differentiation that characteristically involves the scalp and face. It may present as a solitary sporadic lesion or, less frequently, as multiple confluent tumors in the context of CYLD cutaneous syndrome, the unifying designation for Brooke–Spiegler syndrome, familial cylindromatosis and multiple familial trichoepitheliomas. Although histologically benign, multiple scalp cylindromas produce progressive disfigurement, cicatricial alopecia, ulceration and, in a minority of patients, malignant transformation to cylindrocarcinoma, an aggressive neoplasm with documented capacity for local destruction and metastasis. We report a 64-year-old woman with a lifelong history of multiple nodular lesions of the scalp, face and trunk that began in adolescence, in whom a chronically ulcerated frontal lesion raised clinical suspicion of malignant transformation. Wide surgical excision of the frontal and scalp lesions was performed with reconstruction by a full-thickness skin graft harvested from the abdominal region. Histopathological examination demonstrated ulcerated cylindromas with free surgical margins, and graft integration reached approximately 95%. Residual scalp tumors are being managed by planned staged excision in order to preserve as much hair-bearing scalp as possible. Using this case as a framework, we review the historical background, epidemiology, histopathological features, molecular pathogenesis and contemporary management of cutaneous cylindroma, with particular emphasis on the reconstructive decisions that confront the plastic surgeon. Recognition of the clinical warning signs of malignant change, appropriate genetic characterization of the patient and family, and the principle of tissue-preserving staged surgery remain central to the modern care of these patients.