PMID- 23108259 OWN - NLM STAT- MEDLINE DCOM- 20130930 LR - 20171116 IS - 1423-0399 (Electronic) IS - 0042-1138 (Linking) VI - 90 IP - 2 DP - 2013 TI - Primitive neuroectodermal tumor of the kidney: a single-institute series of six patients from China. PG - 174-8 LID - 10.1159/000342647 [doi] AB - OBJECTIVE: Renal primitive neuroectodermal tumor (PNET) is a rare and highly malignant neoplasm. Our study aims to present the clinical findings and prognosis of 6 patients with renal PNET from China. MATERIAL AND METHODS: From January 2003 to February 2012, 6 patients with renal PNET confirmed by immunohistochemical staining and fluorescence in situ hybridization (FISH) treated in our institution were reviewed retrospectively and our data compared with the literature. RESULTS: There were 4 male and 2 female patients with a mean age of 35.0 years. The mean diameter was 13.3 cm. Five of the masses had renal vein thrombosis, and 3 extended into the inferior vena cava. A lymph node metastasis was detected in 1 patient and a liver metastasis in another patient. Radical nephrectomy was performed in all cases, with 5 patients following chemotherapy and 1 receiving Sutent treatment. Within a median follow-up of 7.5 months (range 1-71), median overall survival was 22.1 months. CONCLUSIONS: The diagnosis of renal PNET should be considered in young patients presenting with renal mass and venous thrombosis. It seems that Asian renal PNET patients had a higher rate of venous thrombosis than Caucasian populations. CD99 positivity on immunohistochemistry and EWS-FL1 chimera gene detected by FISH support the diagnosis. The prognosis is poor despite multimodal treatment. CI - Copyright (c) 2012 S. Karger AG, Basel. FAU - Huang, Jiwei AU - Huang J AD - Department of Urology, Renji Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, PR China. FAU - Zhang, Jin AU - Zhang J FAU - Dong, Baijun AU - Dong B FAU - Chen, Yonghui AU - Chen Y FAU - Kong, Wen AU - Kong W FAU - Liu, Qiang AU - Liu Q FAU - Xue, Wei AU - Xue W FAU - Liu, Dongming AU - Liu D FAU - Huang, Yiran AU - Huang Y LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20121026 PL - Switzerland TA - Urol Int JT - Urologia internationalis JID - 0417373 RN - 0 (12E7 Antigen) RN - 0 (Antigens, CD) RN - 0 (CD99 protein, human) RN - 0 (Cell Adhesion Molecules) RN - 0 (EWS-FLI fusion protein) RN - 0 (Oncogene Proteins, Fusion) RN - 0 (Proto-Oncogene Protein c-fli-1) RN - 0 (RNA-Binding Protein EWS) SB - IM MH - 12E7 Antigen MH - Adolescent MH - Adult MH - Aged MH - Antigens, CD/metabolism MH - Cell Adhesion Molecules/metabolism MH - China MH - Female MH - Humans MH - Kidney Neoplasms/*diagnosis/mortality/therapy MH - Lymphatic Metastasis MH - Male MH - Middle Aged MH - Neuroectodermal Tumors, Primitive/*diagnosis/mortality/therapy MH - Oncogene Proteins, Fusion/metabolism MH - Prognosis MH - Proto-Oncogene Protein c-fli-1/metabolism MH - RNA-Binding Protein EWS/metabolism MH - Renal Veins/physiopathology MH - Retrospective Studies MH - Treatment Outcome MH - Vena Cava, Inferior/physiopathology MH - Venous Thrombosis/complications MH - Young Adult EDAT- 2012/10/31 06:00 MHDA- 2013/10/01 06:00 CRDT- 2012/10/31 06:00 PHST- 2012/05/07 00:00 [received] PHST- 2012/08/02 00:00 [accepted] PHST- 2012/10/31 06:00 [entrez] PHST- 2012/10/31 06:00 [pubmed] PHST- 2013/10/01 06:00 [medline] AID - 000342647 [pii] AID - 10.1159/000342647 [doi] PST - ppublish SO - Urol Int. 2013;90(2):174-8. doi: 10.1159/000342647. Epub 2012 Oct 26.