Local staging and treatment in extremity rhabdomyosarcoma. A report from the EpSSG-RMS2005 study

Sheila E.J. Terwisscha van Scheltinga; Marc H.W.A. Wijnen; Hélène Martelli; Timothy Rogers; Henry Mandeville; Mark N. Gaze; Keiran McHugh; Nadege Corradini; Daniel Orbach; Meriel Jenney; Anna Kelsey; Julia Chisholm; Soledad Gallego; Heidi Glosli; Andrea Ferrari; Ilaria Zanetti; Gian Luca De Salvo; Veronique Minard-Colin; Giani Bisogno; Max M. van Noesel; Hans H.M. Merks

doi:10.1002/cam4.3365

Local staging and treatment in extremity rhabdomyosarcoma. A report from the EpSSG-RMS2005 study

Sheila E.J. Terwisscha van Scheltinga, Marc H.W.A. Wijnen, Hélène Martelli, Timothy Rogers, Henry Mandeville, Mark N. Gaze, Keiran McHugh, Nadege Corradini, Daniel Orbach, Meriel Jenney, Anna Kelsey, Julia Chisholm, Soledad Gallego, Heidi Glosli, Andrea Ferrari, Ilaria Zanetti, Gian Luca De Salvo, Veronique Minard-Colin, Giani Bisogno, Max M. van NoeselHans H.M. Merks

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Abstract

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.

Original language	English
Pages (from-to)	7580-7589
Number of pages	10
Journal	Cancer Medicine
Volume	9
Issue number	20
DOIs	https://doi.org/10.1002/cam4.3365
Publication status	Published - 1 Oct 2020

Keywords

local therapy
lymph node metastases
radiotherapy
rhabdomyosarcoma
staging
surgery

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Terwisscha van Scheltinga, S. E. J., Wijnen, M. H. W. A., Martelli, H., Rogers, T., Mandeville, H., Gaze, M. N., McHugh, K., Corradini, N., Orbach, D., Jenney, M., Kelsey, A., Chisholm, J., Gallego, S., Glosli, H., Ferrari, A., Zanetti, I., De Salvo, G. L., Minard-Colin, V., Bisogno, G., ... Merks, H. H. M. (2020). Local staging and treatment in extremity rhabdomyosarcoma. A report from the EpSSG-RMS2005 study. Cancer Medicine, 9(20), 7580-7589. https://doi.org/10.1002/cam4.3365

@article{6686986ea7dd44d7bab9ba9cdba38ddf,

title = "Local staging and treatment in extremity rhabdomyosarcoma. A report from the EpSSG-RMS2005 study",

abstract = "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.",

keywords = "local therapy, lymph node metastases, radiotherapy, rhabdomyosarcoma, staging, surgery",

author = "{Terwisscha van Scheltinga}, {Sheila E.J.} and Wijnen, {Marc H.W.A.} and H{\'e}l{\`e}ne Martelli and Timothy Rogers and Henry Mandeville and Gaze, {Mark N.} and Keiran McHugh and Nadege Corradini and Daniel Orbach and Meriel Jenney and Anna Kelsey and Julia Chisholm and Soledad Gallego and Heidi Glosli and Andrea Ferrari and Ilaria Zanetti and {De Salvo}, {Gian Luca} and Veronique Minard-Colin and Giani Bisogno and {van Noesel}, {Max M.} and Merks, {Hans H.M.}",

note = "Publisher Copyright: {\textcopyright} 2020 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.",

year = "2020",

month = oct,

day = "1",

doi = "10.1002/cam4.3365",

language = "English",

volume = "9",

pages = "7580--7589",

journal = "Cancer Medicine",

issn = "2045-7634",

number = "20",

}

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 2020, 'Local staging and treatment in extremity rhabdomyosarcoma. A report from the EpSSG-RMS2005 study', Cancer Medicine, vol. 9, no. 20, pp. 7580-7589. https://doi.org/10.1002/cam4.3365

TY - JOUR

T1 - Local staging and treatment in extremity rhabdomyosarcoma. A report from the EpSSG-RMS2005 study

AU - Terwisscha van Scheltinga, Sheila E.J.

AU - Wijnen, Marc H.W.A.

AU - Martelli, Hélène

AU - Rogers, Timothy

AU - Mandeville, Henry

AU - Gaze, Mark N.

AU - McHugh, Keiran

AU - Corradini, Nadege

AU - Orbach, Daniel

AU - Jenney, Meriel

AU - Kelsey, Anna

AU - Chisholm, Julia

AU - Gallego, Soledad

AU - Glosli, Heidi

AU - Ferrari, Andrea

AU - Zanetti, Ilaria

AU - De Salvo, Gian Luca

AU - Minard-Colin, Veronique

AU - Bisogno, Giani

AU - van Noesel, Max M.

AU - Merks, Hans H.M.

PY - 2020/10/1

Y1 - 2020/10/1

N2 - 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.

AB - 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.

KW - local therapy

KW - lymph node metastases

KW - radiotherapy

KW - rhabdomyosarcoma

KW - staging

KW - surgery

UR - http://www.scopus.com/inward/record.url?scp=85090002462&partnerID=8YFLogxK

U2 - 10.1002/cam4.3365

DO - 10.1002/cam4.3365

M3 - Article

C2 - 32869534

AN - SCOPUS:85090002462

SN - 2045-7634

VL - 9

SP - 7580

EP - 7589

JO - Cancer Medicine

JF - Cancer Medicine

IS - 20

ER -

Local staging and treatment in extremity rhabdomyosarcoma. A report from the EpSSG-RMS2005 study

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Keywords

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