Nevoid basal cell carcinoma syndrome. Report of a case with associated Hodgkin's disease.

PURPOSE: Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal-dominant multisystem disorder characterized by various congenital anomalies, multiple cutaneous basal cell carcinomas (BCCs), and increased risk for other benign and malignant tumors. We report a 13-year-old girl with NBCCS who developed cutaneous BCCs 2 years after radiotherapy for Hodgkin's lymphoma.

PATIENTS AND

METHODS

Review of clinical and laboratory observations of patients with NBCCS suggest a close relationship between radiotherapy and the early emergence of numerous BCCs. However, in vitro studies of the effect of radiosensitivity on cells cultured from patients with NBCCS show inconsistent results.

RESULTS

Clarification of the role of radiotherapy in the induction of BCCs in NBCCS requires additional studies. Nevertheless, recognition of the syndrome is important because whenever possible radiotherapy for secondary tumors might be effectively replaced by chemotherapy.