title: Primitive neuroectodermal tumor of the kidney: a single-institute series of six patients from China.
abstract: 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.
  
  
  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.
authors.affiliation: Department of Urology, Renji Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, PR China.
authors.forename: Jiwei
authors.initials: J
authors.lastname: Huang
authors.forename: Jin
authors.initials: J
authors.lastname: Zhang
authors.forename: Baijun
authors.initials: B
authors.lastname: Dong
authors.forename: Yonghui
authors.initials: Y
authors.lastname: Chen
authors.forename: Wen
authors.initials: W
authors.lastname: Kong
authors.forename: Qiang
authors.initials: Q
authors.lastname: Liu
authors.forename: Wei
authors.initials: W
authors.lastname: Xue
authors.forename: Dongming
authors.initials: D
authors.lastname: Liu
authors.forename: Yiran
authors.initials: Y
authors.lastname: Huang
mesh_terms: D000071443:12E7 Antigen; D000293:Adolescent; D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000378:metabolism; D015815:Cell Adhesion Molecules / Q000378:metabolism; D002681:China; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D015514:Oncogene Proteins, Fusion / Q000378:metabolism; D011379:Prognosis; D051789:Proto-Oncogene Protein c-fli-1 / Q000378:metabolism; D034802:RNA-Binding Protein EWS / Q000378:metabolism; D012082:Renal Veins / Q000503:physiopathology; D012189:Retrospective Studies; D016896:Treatment Outcome; D014682:Vena Cava, Inferior / Q000503:physiopathology; D020246:Venous Thrombosis / Q000150:complications; D055815:Young Adult
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS