title: Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee.
abstract: BACKGROUND
  To evaluate the effect of radiotherapy (RT) in association with complete second look operation, histologically confirmed, on outcome of patients with IRS Gr.III non-alveolar RMS.
  
  
  METHODS
  We analyzed data from 39 patients (age: 0.5-194 months, median 52) who were enrolled between 1988 and 2005 in 2 consecutive Italian Studies, RMS 88 and RMS 96. All achieved a complete resection of the residual tumor after neoadjuvant chemotherapy; 27 did not receive any other local treatment: pelvic 8, extremities 6, head-neck-non-parameningeal 5, orbit 1, genito-urinary-bladder-prostate 3, trunk 2, abdomen 1, vagina 1; 12 were given RT (32-45 Gy), 5 before and 7 after the operation: genito-urinary-bladder-prostate 3, pelvic 3, abdominal 1, extremities 1, head-neck-parameningeal 1, head-neck-non-parameningeal 1, vagina 1, orbit 1. All received postoperative chemotherapy.
  
  
  RESULTS
  Median follow-up was 81 months (range 17-219 months). With RT: 10/12 patients are in first complete remission; 2/12 had a metastatic relapse (1 also local relapse), and both of them died of disease. Without RT: 16/27 maintained the first complete remission, however 1/16 died due to a second tumor; 8 suffered from local relapse (4 pelvic, 1 orbit, 1 vagina, 1 head-neck-non-parameningeal, 1 abdomen) and 3 of them died, 3 showed a metastatic recurrence (2 extremities, 1 pelvic) and 1 died.
  
  
  CONCLUSIONS
  Local relapses were more frequent for patients without RT, especially in pelvic sites. The two relapses after RT occurred in huge bladder-prostate RMS. Although the limited number of patients does not allow statistically significant conclusions, our experience suggests that RT may have a positive influence on local control for completely resected non-alveolar RMS.
authors.affiliation: Division of Pediatric Surgery, Department of Pediatrics, University Hospital of Padua, Padua, Italy. giovanni.cecchetto@unipd.it
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Elena
authors.initials: E
authors.lastname: Carretto
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Patrizia
authors.initials: P
authors.lastname: Dall'Igna
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Giovanni
authors.initials: G
authors.lastname: Scarzello
authors.forename: Alberto
authors.initials: A
authors.lastname: Donfrancesco
authors.forename: Rita
authors.initials: R
authors.lastname: Alaggio
authors.forename: Paolo
authors.initials: P
authors.lastname: Indolfi
authors.forename: Modesto
authors.initials: M
authors.lastname: Carli
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000628:therapy; D018714:Radiotherapy, Adjuvant; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000532:radiotherapy* / Q000601:surgery*; D012509:Sarcoma; D024162:Second-Look Surgery