OBJECTIVETo review the clinical characteristics, diagnosis and treatments of primitive neuroectodermal tumor (PNET) in head and neck.

METHODSA retrospective review of the medical records for the cases of PNET in head and neck from 2004 to 2014. General clinical information including diagnosis and treatments was obtained and analyzed. A literature review was also conducted.

RESULTSA total of 5 cases diagnosed with PNET were included. Of 5 patients, one patient with lesion in the temporal field was treated with radical resection, followed by radiotherapy, and a 24-month follow-up showed no recurrence. Three patients presented with maxillary, infratemporal fossa or cheek invasion respectively, after diagnosed with pathological examination, one patient received preoperation radiotherapy plus radical resection and postoperative chemotherapy, and the lesions had no obvious progress with follow-up of 106 months; one patient was applied with preoperative chemotherapy plus extensive resection and postoperation radiotherapy, showed recurrence 15 months later, and was lost to follow-up; and another patient underwent chemotherapy plus radiotherapy and extensive resection, presented with pulmonary metastasis 5 months later, and died of brain metastasis within 25 months. One foreign patient presented with tumor involved submaxillary, mouth and tongue, the tumor was reduced obviously after chemotherapy, but he was lost to follow-up after getting home.

CONCLUSIONSPNET in head and neck is rare and the clinical profile of PNET is the presence of occupying and compression. The pathology examination is an only way to confirm the diagnosis of PNET. The combination of excision, radiotherapy and chemotherapy is the treatment choice.