Abstract
Nevoid basal cell carcinoma syndrome (NBCCS) was first reported by Jarisch in 1894. The major symptoms of NBCCS are multiple basal cell carcinomas, multiple jaw cysts, various skeletal malformations, calcification of the falx cerebri, and palmo-plantar pits. We experienced two cases with NBCCS. Case 1: A 18-year-old man had had multiple brown nodules on his scalp for two years. Histopathological examination revealed them to be basal cell carcinoma. CT examination on his scalp showed calcification of the falx cerebri. Additional symptoms supporting the diagnosis of NBCCS were palmar pits and ocular hypertelorism. Case 2 : A 17-year-old man also had multiple basal cell carcinomas, calcification of the falx cerebri, palmar pits, and positive family history. Their symptoms appeared following the medulloblastoma, a relatively unknown manifestation of NBCCS. Only 12 cases of NBCCS with medulloblastoma, including our two cases, have been reported in Japan. Among the clinical features observed in these patients of NBCCS with medulloblastoma, the following are considered to be important: the earlier occurrence of medulloblastoma than in sporadic cases, multiple BCCs of the head following craniospinal radiation for medulloblastoma, and the high incidence of radiation-induced meningioma.
