Therapy and outcome of primitive neuroectodermal tumor of the jaw

Data pertaining to outcomes in jaw primitive neuroectodermal tumor (PNET) are lacking. Eleven cases of jaw PNET (five maxillary and six mandibular) were treated at our cancer center with the same chemotherapeutic agents from June 2003 to January 2009. Four patients underwent surgery after neoadjuvant chemotherapy and all received local radiotherapy. At median follow-up of 56 months (range: 19-77 months), 7/11 patients are in sustained remission. There was no difference in outcome with respect to site of tumor, and whether surgery was performed or not. These results support the use of chemoradiation as initial modality of treatment rather than going for extensive and mutilating surgery.