Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee

Pediatr Blood Cancer. 2008 Nov;51(5):593-7. doi: 10.1002/pbc.21702.

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.

Procedure: 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Child
  • Child, Preschool
  • Clinical Trials as Topic
  • Combined Modality Therapy
  • Female
  • Humans
  • Infant
  • Kaplan-Meier Estimate
  • Male
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / therapy
  • Radiotherapy, Adjuvant
  • Rhabdomyosarcoma / drug therapy
  • Rhabdomyosarcoma / radiotherapy*
  • Rhabdomyosarcoma / surgery*
  • Sarcoma
  • Second-Look Surgery*