Primitive neuroectodermal tumor and its features in Central Nervous System

Objective To elucidate the clinical features and treatment of Primitive neuroectodermal tumor (PNET) in every part of the body, especially in CNS. Methods Clinical records of 89 cases with PNET of every system treated at 301 Hospital between 1996 and 2006 were carefully assembled. A review of relevant literatures was also done. The duration of follow-up was from 4 to 25 months (mean 13 months). Results Among 89 patients of HPC, 21eases (24%) occurred in CNS (9 in cerebral hemisphere; 8 in spinal canal; 4 in brain stem). The rest 68 cases (76%) occurred in other systems. All the 21 cases PNET in CNS underwent tumorectomy. Among all the 25 operations, total tumor removal was achieved in 18 cases and subtotal removal in 7 cases. Local recurrence occurred 12 times (80%) in 15 patients with successful visit. 4 patients (27%) presented one or more distant metastasis, and 10 patients died. Conehlsious PNET in CNS and PNET in other systems displayed similar clinical characteristics and pathological morphous. It is a great challenge to neurosurgeons as well as other surgeons to cure the disease. PNET has an unfavorable prognosis because its high rate of recurrence and metastasis. PNET in CNS as well as in other parts of the body should be recognized, as one disease as a whole. At the same time, PNET in CNS has its distinct features. Key words: Neuroectodermal tumors, primitive;  Pathological conditions, signs and symptoms;  Recurrence