Analysis Of Clinical Characteristics And Treatment Methods In Peripheral Primitive Neuroectodermal Tumors

Objective: To investigate the clinical and pathological characteristics,treatment methods, efficacy and research progress of peripheral primitive neuroectodermal tumors( pPNET) to guide the clinical diagnosis and treatment.Methods: The characteristics of clinical, pathology and treatment methods of 10 cases with pPNET were studied retrospectively in the first affiliated hospital of Chongqing medical university from November 2005 to April 2014 and calculated the survival rate of patients by direct method.The data was calculated by SPSS19.0.Results: The ten cases were mainly male teenagers, in which lump or/and pain were often the primary lesion. On histology and immunohistochemistry, tumor cells strongly expressed CD99 and NSE,Syn and Vim were also seen, S-100, CgA, FLI-1and NF were observed in some cases. 6 patients were treated with surgical resection, in which 5 cases received postoperative radiotherapy and chemotherapy.2 patients received radiotherapy and chemotherapy. 1 patient received chemotherapy and HIFU.1 patient was treated with Chinese Medicine. The 1,2,5-year survival rates of the patients were 80.0%(8/10), 71.4%(5/7) and 40.0%(2/5),respectively.Conclusions: The clinical characteristics of pPNET are lack of specificity. The diagnosis of pPNET mainly depends on histology and immunohistochemistry. The multimodality therapy of pPNET includes surgery, radiotherapy and chemotherapy.