Multiple neoplasms following craniospinal irradiation for medulloblastoma in a patient with nevoid basal cell carcinoma syndrome. Case report.

Abstract

A 28-year-old man presented to the authors' hospital with multiple intracranial tumors. At 2 years of age, he had undergone resection of a medulloblastoma and received adjunctive craniospinal irradiation. Subsequently, he was diagnosed with nevoid basal cell carcinoma syndrome, Gorlin's syndrome. Since his first presentation, he has required surgery for multiple basal cell carcinomas, an osteochondroma of the rib, two meningiomas, a trigeminal schwannoma, and a pleomorphic liposarcoma, all of which arose within the radiation field. Despite this impressive list of benign and malignant neoplasms, the patient is relatively well and leads a normal life. The authors examine the relationships between Gorlin's syndrome and radiation therapy and the subsequent development of tumors.