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

Am J Otolaryngol. 2007 Sep-Oct;28(5):360-2. doi: 10.1016/j.amjoto.2006.10.006.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Basal Cell Nevus Syndrome / complications*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Medulloblastoma / radiotherapy
  • Neoplasms, Radiation-Induced / diagnosis
  • Neoplasms, Radiation-Induced / surgery*
  • Paranasal Sinus Neoplasms / diagnosis
  • Paranasal Sinus Neoplasms / etiology*
  • Paranasal Sinus Neoplasms / surgery*
  • Radiotherapy / adverse effects*