title: Clear cell sarcomas of the kidney registered on International Society of Pediatric Oncology (SIOP) 93-01 and SIOP 2001 protocols: a report of the SIOP Renal Tumour Study Group.
abstract: OBJECTIVE
  Clear Cell Sarcoma of the Kidney (CCSK) is a rare childhood renal tumour. Only a few homogeneously treated CCSK cohorts have been reported. This study aims to describe clinical characteristics and survival of CCSK patients treated according to recent International Society of Pediatric Oncology (SIOP) protocols.
  
  
  METHODS
  We analysed the prospectively collected data of patients with a histologically verified CCSK, entered onto SIOP 93-01/2001 trials.
  
  
  RESULTS
  A total of 191 CCSK patients (64% male) were analysed, with a median age at diagnosis of 2.6 years. Stage distribution for stages I, II, III and IV was 42%, 23%, 28% and 7%, respectively. Pre-operative chemotherapy was administered to 169/191 patients. All patients underwent total nephrectomy and 189/191 patients received post-operative chemotherapy. Radiotherapy was applied in 2/80 stage I, 33/44 stage II, 44/54 stage III and 6/13 stage IV patients. Five year event-free survival (EFS) and overall survival (OS) were 79% (95% confidence interval (CI): 73-85%) and 86% (95% CI: 80-92%) respectively. Stage IV disease and young age were significant adverse prognostic factors for event-free survival. Factors such as gender, tumour volume and type of initial treatment were not found to be prognostic for EFS and OS.
  
  
  CONCLUSIONS
  In this largest SIOP cohort described so far, overall outcome of CCSK is reasonable, although treatment of young and advanced-stage disease patients is challenging. As further intensification of treatment is hampered by direct and late toxicity, future directions should include the development of targeted therapy based on specific molecular aberrations of CCSK.
authors.affiliation: Department of Pediatric Hematology/Oncology, University Hospital for Children, Homburg, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Furtwängler
authors.forename: S L
authors.initials: SL
authors.lastname: Gooskens
authors.forename: H
authors.initials: H
authors.lastname: van Tinteren
authors.forename: J
authors.initials: J
authors.lastname: de Kraker
authors.forename: G
authors.initials: G
authors.lastname: Schleiermacher
authors.forename: C
authors.initials: C
authors.lastname: Bergeron
authors.forename: B
authors.initials: B
authors.lastname: de Camargo
authors.forename: T
authors.initials: T
authors.lastname: Acha
authors.forename: J
authors.initials: J
authors.lastname: Godzinski
authors.forename: B
authors.initials: B
authors.lastname: Sandstedt
authors.forename: I
authors.initials: I
authors.lastname: Leuschner
authors.forename: G M
authors.initials: GM
authors.lastname: Vujanic
authors.forename: R
authors.initials: R
authors.lastname: Pieters
authors.forename: N
authors.initials: N
authors.lastname: Graf
authors.forename: M M
authors.initials: MM
authors.lastname: van den Heuvel-Eibrink
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D007680:Kidney Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D020360:Neoadjuvant Therapy; D009367:Neoplasm Staging; D009392:Nephrectomy; D016016:Proportional Hazards Models; D018714:Radiotherapy, Adjuvant; D012307:Risk Factors; D018227:Sarcoma, Clear Cell / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D013997:Time Factors; D016896:Treatment Outcome
keywords: Clear Cell Sarcoma of the Kidney; Clinical features; Histology; Outcome; Treatment