title: Challenges in the local treatment of large abdominal embryonal rhabdomyosarcoma.
abstract: BACKGROUND
  Embryonal rhabdomyosarcoma is the most common pediatric soft tissue sarcoma. The best local treatment in large, nonmetastatic primary unresected nongenitourinary embryonal rhabdomyosarcoma of the abdomen (LARME) is however unclear.
  
  
  METHODS
  We analyzed patients with LARME treated in four consecutive CWS trials. All diagnoses were confirmed by reference reviews. Treatment included multiagent chemotherapy and local treatment of the primary tumor with surgery and/or radiotherapy. The impact of primary debulking surgery (PDS) also was studied.
  
  
  RESULTS
  One hundred patients <21 years with a median age of 4 years had LARME. Sixty-one of them had a tumor >10 cm in diameter at diagnosis. PDS was performed in 19 of 100 children. The outcomes of patients with PDS were similar to those of the other patients. In 36 children, the tumor was resected after induction chemotherapy; 60 RME were irradiated. The toxic effects of radiochemotherapy were not significantly increased compared with the nonirradiated patients. With a median follow-up of 10 years, the 5-year EFS and OS were 52 ± 10 and 65 ± 9 %, respectively. Significant risk factors in multivariate analysis were age >10 years; no achievement of complete remission; and inadequate secondary local treatment, defined as incomplete secondary resection or no radiation.
  
  
  CONCLUSIONS
  Children with LARME have a fair prognosis, despite an often huge tumor size and unfavorable primary site, if the tumors can either be resected or irradiated following induction chemotherapy. PDS was only performed in a small subgroup. Radiation performed concomitantly with chemotherapy did not increase the acute toxicity significantly.
authors.affiliation: Pediatrics 5 (Oncology, Hematology, Immunology, Rheumatology, Gastroenterology and General Pedatrics), Olgahospital, Klinikum Stuttgart, Stuttgart, Germany, tobias.dantonello@olgahospital-stuttgart.de.
authors.forename: Tobias M
authors.initials: TM
authors.lastname: Dantonello
authors.forename: Helmut
authors.initials: H
authors.lastname: Lochbühler
authors.forename: Andreas
authors.initials: A
authors.lastname: Schuck
authors.forename: Stefanie
authors.initials: S
authors.lastname: Kube
authors.forename: Jan
authors.initials: J
authors.lastname: Godzinski
authors.forename: Erik
authors.initials: E
authors.lastname: Sköldenberg
authors.forename: Gustaf
authors.initials: G
authors.lastname: Ljungman
authors.forename: Daniel
authors.initials: D
authors.lastname: Kosztyla
authors.forename: Iris
authors.initials: I
authors.lastname: Veit-Friedrich
authors.forename: Erika
authors.initials: E
authors.lastname: Hallmen
authors.forename: Simone
authors.initials: S
authors.lastname: Feuchtgruber
authors.forename: Ruediger
authors.initials: R
authors.lastname: Wessalowski
authors.forename: Markus
authors.initials: M
authors.lastname: Franke
authors.forename: Stefan S
authors.initials: SS
authors.lastname: Bielack
authors.forename: Thomas
authors.initials: T
authors.lastname: Klingebiel
authors.forename: Ewa
authors.initials: E
authors.lastname: Koscielniak
mesh_terms: D000008:Abdominal Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D012074:Remission Induction; D018233:Rhabdomyosarcoma, Embryonal / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D015996:Survival Rate; D055815:Young Adult