The nevoid basal-cell carcinoma syndrome is a rare inherited dermatological disease characterised by the early onset of multiple basal-cell carcinomas, palmo-plantar pitting and by the involvement of the ocular, endocrine and central nervous systems. We report herein one such case with medulloblastoma as first presentation of the disease. After a latency of 17 years, the patient presented multiple basal-cell carcinomas, some of which located in skin areas corresponding to the ports of irradiation treatment for medulloblastoma. It was not possible to ascertain whether the growth of these neoplasms was due to genetic features or to the past radiation treatment. Probably, the combination of the two factors was determinant. The finding of low pitched female voice, hypogonadism, mental retardation and meningioma supported the diagnosis of nevoid basal-cell carcinoma syndrome. Cautious treatment of the basal-cell carcinomas with superficial radiotherapy was performed with success.
|Number of pages||4|
|Publication status||Published - Jul 2003|
- Basal-cell carcinoma
- Gorlin-Goltz syndrome
- Nevoid basal-cell carcinoma syndrome
ASJC Scopus subject areas