title: Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies.
abstract: OBJECTIVE
  Peripheral primitive neuroectodermal tumor (PNET) is a highly aggressive neoplasm belonging to the Ewing family of tumors. It is characterized by the expression of MIC2 and neural markers (eg, neuron-specific enolase, synaptophysin, S-100, vimentin, Leu-7), and the presence of the EWS-FLI1 translocation.
  
  
  METHODS
  We performed a MEDLINE search for PNET in urologic malignancies. Additionally, we report on 2 cases of renal and 1 case of bladder PNET. The data obtained by reviewing patients with renal PNET were analyzed using Kaplan-Meier analysis.
  
  
  RESULTS
  Renal PNET is diagnosed in young adults (median age 24 years). In contrast, the incidence of bladder PNET seems to be dependent on a defective immune mechanism. Patients often present with pain (84%), palpable tumor (60%), and hematuria (38%). The radiologic findings are uncharacteristic; therefore, the diagnosis should be based on the histologic and immunohistochemistry findings. Renal and bladder PNET are both often diagnosed at an advanced stage and, therefore, the prognosis is poor, despite aggressive multimodal treatment (surgery, polychemotherapy, radiotherapy). We identified palpable tumor masses (log-rank test, P = 0.0027) and synaptophysin expression (log-rank test, P = 0.0422) as prognostic unfavorable markers for renal PNET.
  
  
  CONCLUSIONS
  Renal PNET should be considered in young patients who present with the classic triad of renal cancer, hematuria, and pain and palpable tumor. Once PNET is diagnosed, multimodal treatment (radical surgery, multidrug chemotherapy, radiotherapy) must be initiated. Despite this, the prognosis is poor if distant metastases are present. Furthermore, palpable tumor masses and synaptophysin expression are associated with a shorter cancer-specific survival.
authors.affiliation: Klinik und Poliklinik für Urologie, Universitätsklinikum Bonn, Rheinische Friedrich-Wilhelms-Universität Bonn, Bonn, Germany.
authors.forename: Jörg
authors.initials: J
authors.lastname: Ellinger
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: Bastian
authors.forename: Stefan
authors.initials: S
authors.lastname: Hauser
authors.forename: Katharina
authors.initials: K
authors.lastname: Biermann
authors.forename: Stefan C
authors.initials: SC
authors.lastname: Müller
mesh_terms: D000328:Adult; D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000628:therapy; D008297:Male; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis* / Q000628:therapy; D001749:Urinary Bladder Neoplasms / Q000175:diagnosis / Q000628:therapy; D014571:Urologic Neoplasms / Q000175:diagnosis* / Q000628:therapy