title: Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging: Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies.
abstract: OBJECTIVE
  To report the results from International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors studies (MMT 89 and 95) of males with nonmetastatic paratesticular rhabdomyosarcoma.
  
  
  METHODS
  From 1989 to 2003, 159 patients were included. Radical inguinal orchidectomy was recommended, but retroperitoneal lymph node (LN) assessment was based on imaging alone. The treatment was stratified by stage (SIOP tumor-node-metastasis staging system) and histology.
  
  
  RESULTS
  Median age at presentation was 5.6 years (range 0.3-17.6) and 120 patients were of <10 years (75%). Patients ≥10 years had tumors of >5 cm more frequently compared to patients of <10 years (54% vs. 22%, P = 0.0004). The 5- year overall and progression-free survivals were 94% and 83%, respectively. Seventy-eight percent of relapses occurred in the retroperitoneal LN. Thirty-one percent of stage N0 patients of age ≥10 years developed node relapse, compared with 8% of N0 patients aged <10 years (P = 0.0005).
  
  
  CONCLUSIONS
  Older patients with paratesticular rhabdomyosarcoma have a significant risk of LN relapse. These results support a surgical approach to LN staging in this subgroup of patients.
authors.affiliation: Department of Paediatric Surgery, University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom.
authors.forename: Timothy
authors.initials: T
authors.lastname: Rogers
authors.affiliation: Departement d'Oncologie de l'Enfant et l'Adolescent Institut Gustave Roussy, Villejuif, France.
authors.forename: Veronique
authors.initials: V
authors.lastname: Minard-Colin
authors.affiliation: Clinical Research Unit, Agence de la biomedecine Institut Gustave Roussy, Villejuif, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Cozic
authors.affiliation: Department of Pediatric Oncology, Children's Hospital for Wales Heath Park, Cardiff, United Kingdom.
authors.forename: Meriel
authors.initials: M
authors.lastname: Jenney
authors.affiliation: Department of Pediatric Oncology Emma Children's Hospital-Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: Johannes H M
authors.initials: JHM
authors.lastname: Merks
authors.affiliation: Department of Pediatric Oncology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
authors.forename: Soledad
authors.initials: S
authors.lastname: Gallego
authors.affiliation: Hôpital Universitaire des Enfants Reine Av. J. J. Crocq, Bruxelles, Belgium.
authors.forename: Christine
authors.initials: C
authors.lastname: Devalck
authors.affiliation: Department of Oncology, University College Hospital, London, United Kingdom.
authors.forename: Mark N
authors.initials: MN
authors.lastname: Gaze
authors.affiliation: Department of Pediatric Histopathology, Royal Manchester Children's Hospital, Manchester, United Kingdom.
authors.forename: Anna
authors.initials: A
authors.lastname: Kelsey
authors.affiliation: Departments of Pediatrics, Biostatistics, and Radiation Therapy, Institut, Gustave Roussy, Villejuif, France.
authors.forename: Odile
authors.initials: O
authors.lastname: Oberlin
authors.affiliation: TYA South West Cancer Service, Bristol Haematology Oncology Centre, Bristol, United Kingdom.
authors.forename: Mike
authors.initials: M
authors.lastname: Stevens
authors.affiliation: Department of Paediatric Surgery, University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom.
authors.forename: Richard D
authors.initials: RD
authors.lastname: Spicer
authors.affiliation: Institut d'hématologie et d'Oncologie Pédiatrique, Centre Léon Bérard, Lyon, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Bergeron
authors.affiliation: Chirurgie Pédiatrique Hôpital Bicêtre, Université Paris XI, Orsay, France.
authors.forename: Helene
authors.initials: H
authors.lastname: Martelli
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008207:Lymphatic Metastasis / Q000175:diagnosis; D008297:Male; D009367:Neoplasm Staging / Q000379:methods*; D009919:Orchiectomy / Q000379:methods; D012186:Retroperitoneal Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D012611:Scrotum / Q000601:surgery; D055815:Young Adult
keywords: lymph nodes; paratesticular; retroperitoneal; rhabdomyosarcoma; staging