Sinonasal carcinoma after irradiation for medulloblastoma in nevoid basal cell carcinoma syndrome.

Background: Nevoid basal cell carcinoma syndrome (NBCCS) is associated with multiple basal cell carcinomas, odontogenic cysts, craniofacial anomalies, and childhood medulloblastomas. In addition, it has been associated with irradiation-induced neoplasms including, meningiomas, sarcomas, and gliomas.

Methods: We present a 19-year-old man with NBCCS who presented with a sinonasal carcinoma 17 years after receiving craniospinal irradiation for treatment of medulloblastoma.

Results: To our knowledge, this is the first report of a sinonasal tumor after irradiation in a patient with NBCCS.

Conclusions: With this case, the authors examine the genotype of NBCCS patients and their propensity for radiation-induced tumors. In addition, the management of neoplasms in these tumor-sensitive patients is reviewed.