Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee.
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.
Cecchetto
Giovanni
G
Division of Pediatric Surgery, Department of Pediatrics, University Hospital of Padua, Padua, Italy. giovanni.cecchetto@unipd.it
Carretto
Elena
E
Bisogno
Gianni
G
Dall'Igna
Patrizia
P
Ferrari
Andrea
A
Scarzello
Giovanni
G
Donfrancesco
Alberto
A
Alaggio
Rita
R
Indolfi
Paolo
P
Carli
Modesto
M
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