Clinical study of peripheral primitive neuroectodermal tumors of the spinal cord

Objective To investigate the clinical characteristics of the peripheral primitive neuroectodermal tumors (pPNET) of the spinal cord.Method Four cases of pPNET of the spinal cord were treated in our hospital between Nov.2006 and Mar.2010.The clinical characteristics and outcome of them were reviewed.Results There were 1 male and 3 female patients.The age of presentation ranged from 4 to 20 years.Neck hurt was the first clinical symptom,and the symptoms developed rapidly.The preoperative images were impressing.All 4 lesions were given operations,and one lesion was totally removed,2 lesions were subtotally resected,and one was grossly removed.Two patients were dead at last follow - up.Conclusions pPNETs of the spinal cord are rare lesions occuring in young adults.Immunocytochemical staining(CD99)offers an easy way of making a differential diagnosis between cPNET and pPNET.Surgery combined with chemo - radiotherapy is the first treatment choice.The lesions carry a poor prognosis. Key words: Neuroectodermal tumors, primitive;  Spinal cord neoplasms ;  Treatment;  Prognosis