Peripherd Primitive Neuroectodermal Tumors: A Report Of 7 Cases And A Review Of Literature

Objective:To make a further understanding of peripheral primitive neuroectodermal tumor(pPNET).Materials and Methods:Sevenpatients with pPNET(l man and 6 women) were studied by light microscope and immunohistochemisty.Range of age was from 11 to 33 years old(mean 21.2).The primary sites of involvement were the ilium(2 cases),humerus(2 cases),tibia(l case),thoracic vertebra(l case) and lumbar vertebra(l case).X-ray films showed bone osteolytic destruction and soft tissue mass.Surgery were performed in the patients.5 patients received combined chemotherapy and 1 received radiotherapy.Result:The duration of follow up was 3 to 44 months(mean 11.3 months), three patients died of metastatic tumor of lung 16,13,and 11 months after invasion.Conclusion:PPNET was malignant small cell tumor,commonly occurred in children and adolescence.Thorax, paraspinal regions, and limbs were the sites of predilection.The diagnosis of pPNET is difficult to make solely by imaging features. Confirmatory diagnosis is based on pathologic examination .The treatment of pPNET awaits further study.