MULTIPLE NEOPLASMS FOLLOWING CRANIOSPINAL IRRADIATION FOR MEDULLOBLASTOMA IN A PATIENT WITH NEVOID BASAL-CELL CARCINOMA SYNDROME - CASE-REPORT

A 28-year-old man presented to the authors' hospital with multiple int racranial tumors. At 2 years of age, he had undergone resection of a m edulloblastoma and received adjunctive craniospinal irradiation. Subse quently, he was diagnosed with nevoid basal cell carcinoma syndrome, G orlin's syndrome. Since his first presentation, he has required surger y for multiple basal cell carcinomas, an osteochondroma of the rib, tw o meningiomas, a trigeminal schwannoma, and a pleomorphic liposarcoma, all of which arose within the radiation field. Despite this impressiv e list of benign and malignant neoplasms, the patient is relatively we ll and leads a normal life. The authors examine the relationships betw een Gorlin's syndrome and radiation therapy and the subsequent develop ment of tumors.