Clinicopathological study of primary renal primitive neuroectodermal tumor

Objective To investigate the clinicopathological feature, immunological phenotype, therapy and prognosis of primary renal primitive neuroectodermal tumor (primitive neuroectodermal tumor, PNET). Method To report diagnose and treat condition of a child with renal PNET and review relevant literature. Result The male patient aged 13 years old. Color-ultrasound found 3.5 cm × 3.0cm mass of left kidney; CT scan showed edge-cleared equal low density shadows. Before the operation, implement aspiration biopsy under the guidance of CT, and the pathological report showed the Wilms' tumor; implement retroperitoneal left radical nephrectomy. Under light microscope, the formats of oncocyte are consistent that small round or oval shape. The oncocyte was separated into solid sheet or nest by fibrillar connective tissue, form Homer-Wright rosettes and caryokinesis was common. Immunohistochemical markers: CD99, synaptophysin and vimentin were positive, and all diagnosed as PNET. Implement chemotherapy after operation, and followed up for 8 months without recurrence. Conclusion Renal PNET is a rare highly aggressive soft-tissue tumor, which has specific clinicopathologic features and expresses MIC2 gene product CD99. Diagnosis is mainly based on pathomorphological features and immunohistochemical markers. Main treat method is the combination of excision with radiotherapy and chemotherapy. [Ruijin Zhou, Tao Du, Zhonghua Liu, Xiangyang Wang. Clinicopathological study of primary renal primitive neuroectodermal tumor. Life Sci J 2014; 11(1): 312-316]. (ISSN:1097-8135). http://www.lifesciencesite.com 47