Peripheral Primitive Neuroectodermal Tumors of the Head and Neck

OBJECTIVE: Primitive neuroectodermal tumor is a malignant small round cell tumor of neuroectodermal origin. The occurrence of these tumors in the head and neck is rare. Our objective is to document the frequency of presentation, methods of diagnosis, and forms of treatment used to combat primitive neuroectodermal tumors in the head and neck. DESIGN: Retrospective chart review. RESULTS: The most common location for primitive neuroectodermal tumors in our series was the thoracopulmonary region (46%); the next most common location was the head and neck (42%). Metastatic disease was present in 31% of patients at the time of diagnosis. Cytogenic analysis was found to be helpful in confirming the diagnosis of primitive neuroectodermal tumor. Therapy for primitive neuroectodermal tumors included surgery, chemotherapy, and radiation therapy. Survival statistics were poor, with 65% of the patients alive at 2 years' follow-up. CONCLUSIONS: Primitive neuroectodermal tumor is an aggressive malignant small round cell tumor that may present in the head and neck. Long-term survival for patients with primitive neuroectodermal tumor is still poor; however, the use of improved chemotherapeutic agents combined with aggressive surgical control of primary disease and, in some cases, radiation therapy should lead to improved long-term survival.