title: Renal Ewing tumors.
abstract: BACKGROUND
  Renal Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is extremely rare. Clinical symptoms are nonspecific presenting abdominal pain, palpable mass, and hematuria. Owing to advanced technology demonstrating the ES-specific EWS/ETS translocation, this differential diagnosis has become feasible.
  
  
  METHODS
  The German database of GPOH Ewing's sarcoma trials from 1980 to 2009 was searched for kidney as primary site. Twenty-four patients were identified and analyzed. The median time of observation was 3.71 years (range 0.27-8.75 years). Additionally, we carried out a Medline search for renal ES/PNET.
  
  
  RESULTS
  The median age was 24.9 years (range 11-60 years). In 37.5%, patients presented with primary metastases. Tumor thrombi in the adjacent renal vessels occurred in 56.2%. In 90.9%, rearrangements of t(11;22) were found. All patients received a combined chemotherapy according to the EURO-E.W.I.N.G.99 protocol. In accordance, local control consisted predominantly of combined modality surgery and radiation (47%). At 3 years, overall survival (OS) was 0.80 (SE = 0.09), and event-free survival (EFS) 0.66 (SE = 0.11).
  
  
  CONCLUSIONS
  ES/PNET should be considered in the differential diagnosis of renal tumors. Patients with renal ES/PNET respond to and benefit from conventional ES treatment according to ES study protocols. Therefore, an accurate diagnostic approach and a guideline-adapted therapy should be facilitated.
authors.affiliation: Department of Pediatric Hematology and Oncology, University Hospital, Muenster, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Zöllner
authors.forename: U
authors.initials: U
authors.lastname: Dirksen
authors.forename: H
authors.initials: H
authors.lastname: Jürgens
authors.forename: A
authors.initials: A
authors.lastname: Ranft
mesh_terms: D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000188:drug therapy* / Q000532:radiotherapy / Q000601:surgery; D002648:Child; D002986:Clinical Trials as Topic; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000532:radiotherapy / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy* / Q000532:radiotherapy / Q000601:surgery; D012512:Sarcoma, Ewing / Q000188:drug therapy* / Q000532:radiotherapy / Q000601:surgery; D013534:Survival; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents
keywords: Ewing's sarcoma; PNET; kidney; outcome