Local staging and treatment in extremity rhabdomyosarcoma. A report from the EpSSG-RMS2005 study.
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ICR Authors
Authors
Terwisscha van Scheltinga, SEJ
Wijnen, MHWA
Martelli, H
Rogers, T
Mandeville, H
Gaze, MN
McHugh, K
Corradini, N
Orbach, D
Jenney, M
Kelsey, A
Chisholm, J
Gallego, S
Glosli, H
Ferrari, A
Zanetti, I
De Salvo, GL
Minard-Colin, V
Bisogno, G
van Noesel, MM
Merks, HHM
Wijnen, MHWA
Martelli, H
Rogers, T
Mandeville, H
Gaze, MN
McHugh, K
Corradini, N
Orbach, D
Jenney, M
Kelsey, A
Chisholm, J
Gallego, S
Glosli, H
Ferrari, A
Zanetti, I
De Salvo, GL
Minard-Colin, V
Bisogno, G
van Noesel, MM
Merks, HHM
Document Type
Journal Article
Date
2020-10-01
Date Accepted
2020-07-20
Abstract
UNLABELLED: Rhabdomyosarcoma of the extremities present with two main challenges: correct evaluation of initial regional nodal involvement and define adequate local treatment. METHODS: Pediatric patients with localized rhabdomyosarcoma of the extremity included in the EpSSG-RMS2005 study between 2005 and 2014 were evaluated for staging, treatment, and survival. The outcome was compared to the preceding European SIOP-MMT studies. RESULTS: Of the 162 patients included, histology was unfavorable in 113 (70%), 124 (77%) were younger than 10 years, 128 (79%) were IRS III, and 47 (29%) were node-positive. A regional node biopsy was performed in 97 patients (60%) and modified the lymph node stage in 15/97 (16%). Primary and delayed surgery was performed in 155 (96%) and radiotherapy delivered in 118 (73%) patients. Relapse occurred in 61 cases (38%), local in 14 (23%), regional in 13 (21%), distant in 22 (36%), and combined relapse in 12 (20%) with five progressive diseases (8%) and four secondary tumors (7%). Five-year event free (EFS) and overall survival (OS) were 58.4% (95%CI, 50.3-65.7) and 71.7% (63.6-78.4), respectively. In the previous studies MMT89 and MMT95, tumor surgery was performed in 32/53 (60%) and 74/82(90%), respectively, and radiotherapy delivered in 13/53 (25%) and 26/82 (30%), respectively. Five-year EFS and OS were 35.6%, and 50.3% in MMT89 and 54.3% and 68.2% in the MMT95 study. CONCLUSIONS: Even if the lymph node staging was not always complete according to the RMS2005 protocol, node sampling changed lymph node status in a significant number of patients. Despite the higher rate of patients treated with locoregional radiotherapy, survival in RMS2005 did not improve compared to the previous European SIOP-MMT95 study.
Citation
Cancer medicine, 2020, 9 (20), pp. 7580 - 7589
Source Title
Publisher
WILEY
ISSN
2045-7634
eISSN
2045-7634
Research Team
Sarcoma Clinical Trials in children and young people
Paediatric and Adolescent Radiotherapy
Paediatric and Adolescent Radiotherapy