Alveolar rhabdomyosarcoma with regional nodal involvement: Results of a combined analysis from two cooperative groups

European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group

doi:10.1002/pbc.28832

Alveolar rhabdomyosarcoma with regional nodal involvement: Results of a combined analysis from two cooperative groups

European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group

Group Merks

Research output: Contribution to journal › Article › peer-review

27 Citations (Scopus)

Abstract

BACKGROUND: Treatment of children and adolescents with alveolar rhabdomyosarcoma (ARMS) and regional nodal involvement (N1) have been approached differently by North American and European cooperative groups. In order to define a better therapeutic strategy, we analyzed two studies conducted between 2005 and 2016 by the European paediatric Soft tissue sarcoma Study Group (EpSSG) and Children's Oncology Group (COG).

METHODS: We retrospectively identified patients with ARMS N1 enrolled in either EpSSG RMS2005 or in COG ARST0531. Chemotherapy in RMS2005 comprised ifosfamide + vincristine + dactinomycin + doxorubicin (IVADo), IVA and maintenance (vinorelbine, cyclophosphamide); in ARST0531, it consisted of either vincristine + dactinomycin + cyclophosphamide (VAC) or VAC alternating with vincristine + irinotecan (VI). Local treatment was similar in both protocols.

RESULTS: The analysis of the clinical characteristics of 239 patients showed some differences between study groups: in RMS2005, advanced Intergroup Rhabdomyosarcoma Study Group (IRS) and large tumors predominated. There were no differences in outcomes between the two groups: 5-year event-free survival (EFS), 49% (95% confidence interval [CI]: 39-59) and 44% (95% CI: 30-58), and overall survival (OS), 51% (95% CI: 41-61) and 53.6% (95% CI: 40-68) in RMS2005 and ARST0531, respectively. In RMS2005, EFS of patients with FOXO1-positive tumors was significantly inferior to those with FOXO1-negative (49.3% vs 73%, P = .034). In contrast, in ARST0531, EFS of patients with FOXO1-positive tumors was 45% compared with 43.8% for those with FOXO1-negative.

CONCLUSIONS: The outcome of patients with ARMS N1 was similar in both protocols. However, patients with FOXO1 fusion-negative tumors enrolled in RMS2005 showed a significantly better outcome, suggesting that different strategies of chemotherapy may have an impact in the outcome of this subgroup of patients.

Original language	English
Article number	e28832
Pages (from-to)	e28832
Journal	Pediatric blood & cancer
Volume	68
Issue number	3
DOIs	https://doi.org/10.1002/pbc.28832
Publication status	Published - Nov 2020

Keywords

Antineoplastic Combined Chemotherapy Protocols/therapeutic use
Child
Child, Preschool
Female
Follow-Up Studies
Humans
Infant
Infant, Newborn
Lymph Nodes/pathology
Male
Prognosis
Retrospective Studies
Rhabdomyosarcoma, Alveolar/drug therapy
Survival Rate

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10.1002/pbc.28832

Cite this

@article{682ed8fbeac441e89314d7b548ae6c1c,

title = "Alveolar rhabdomyosarcoma with regional nodal involvement: Results of a combined analysis from two cooperative groups",

abstract = "BACKGROUND: Treatment of children and adolescents with alveolar rhabdomyosarcoma (ARMS) and regional nodal involvement (N1) have been approached differently by North American and European cooperative groups. In order to define a better therapeutic strategy, we analyzed two studies conducted between 2005 and 2016 by the European paediatric Soft tissue sarcoma Study Group (EpSSG) and Children's Oncology Group (COG).METHODS: We retrospectively identified patients with ARMS N1 enrolled in either EpSSG RMS2005 or in COG ARST0531. Chemotherapy in RMS2005 comprised ifosfamide + vincristine + dactinomycin + doxorubicin (IVADo), IVA and maintenance (vinorelbine, cyclophosphamide); in ARST0531, it consisted of either vincristine + dactinomycin + cyclophosphamide (VAC) or VAC alternating with vincristine + irinotecan (VI). Local treatment was similar in both protocols.RESULTS: The analysis of the clinical characteristics of 239 patients showed some differences between study groups: in RMS2005, advanced Intergroup Rhabdomyosarcoma Study Group (IRS) and large tumors predominated. There were no differences in outcomes between the two groups: 5-year event-free survival (EFS), 49\% (95\% confidence interval [CI]: 39-59) and 44\% (95\% CI: 30-58), and overall survival (OS), 51\% (95\% CI: 41-61) and 53.6\% (95\% CI: 40-68) in RMS2005 and ARST0531, respectively. In RMS2005, EFS of patients with FOXO1-positive tumors was significantly inferior to those with FOXO1-negative (49.3\% vs 73\%, P = .034). In contrast, in ARST0531, EFS of patients with FOXO1-positive tumors was 45\% compared with 43.8\% for those with FOXO1-negative.CONCLUSIONS: The outcome of patients with ARMS N1 was similar in both protocols. However, patients with FOXO1 fusion-negative tumors enrolled in RMS2005 showed a significantly better outcome, suggesting that different strategies of chemotherapy may have an impact in the outcome of this subgroup of patients.",

keywords = "Antineoplastic Combined Chemotherapy Protocols/therapeutic use, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Lymph Nodes/pathology, Male, Prognosis, Retrospective Studies, Rhabdomyosarcoma, Alveolar/drug therapy, Survival Rate",

author = "\{European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group\} and Soledad Gallego and Yueh-Yun Chi and \{De Salvo\}, \{Gian Luca\} and Minjie Li and Merks, \{Johannes H M\} and Rodeberg, \{David A\} and \{van Scheltinga\}, \{Sheila Terwisscha\} and Leo Mascarenhas and Daniel Orbach and Meriel Jenney and Lynn Million and Veronique Minard-Colin and Suzanne Wolden and Ilaria Zanetti and Parham, \{David M\} and Henry Mandeville and Rajkumar Venkatramani and Gianni Bisogno and Hawkins, \{Douglas S\}",

note = "{\textcopyright} 2020 Wiley Periodicals LLC.",

year = "2020",

month = nov,

doi = "10.1002/pbc.28832",

language = "English",

volume = "68",

pages = "e28832",

journal = "Pediatric blood \& cancer",

issn = "1545-5009",

publisher = "Wiley-Liss Inc.",

number = "3",

}

TY - JOUR

T1 - Alveolar rhabdomyosarcoma with regional nodal involvement

T2 - Results of a combined analysis from two cooperative groups

AU - European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group

AU - Gallego, Soledad

AU - Chi, Yueh-Yun

AU - De Salvo, Gian Luca

AU - Li, Minjie

AU - Merks, Johannes H M

AU - Rodeberg, David A

AU - van Scheltinga, Sheila Terwisscha

AU - Mascarenhas, Leo

AU - Orbach, Daniel

AU - Jenney, Meriel

AU - Million, Lynn

AU - Minard-Colin, Veronique

AU - Wolden, Suzanne

AU - Zanetti, Ilaria

AU - Parham, David M

AU - Mandeville, Henry

AU - Venkatramani, Rajkumar

AU - Bisogno, Gianni

AU - Hawkins, Douglas S

PY - 2020/11

Y1 - 2020/11

N2 - BACKGROUND: Treatment of children and adolescents with alveolar rhabdomyosarcoma (ARMS) and regional nodal involvement (N1) have been approached differently by North American and European cooperative groups. In order to define a better therapeutic strategy, we analyzed two studies conducted between 2005 and 2016 by the European paediatric Soft tissue sarcoma Study Group (EpSSG) and Children's Oncology Group (COG).METHODS: We retrospectively identified patients with ARMS N1 enrolled in either EpSSG RMS2005 or in COG ARST0531. Chemotherapy in RMS2005 comprised ifosfamide + vincristine + dactinomycin + doxorubicin (IVADo), IVA and maintenance (vinorelbine, cyclophosphamide); in ARST0531, it consisted of either vincristine + dactinomycin + cyclophosphamide (VAC) or VAC alternating with vincristine + irinotecan (VI). Local treatment was similar in both protocols.RESULTS: The analysis of the clinical characteristics of 239 patients showed some differences between study groups: in RMS2005, advanced Intergroup Rhabdomyosarcoma Study Group (IRS) and large tumors predominated. There were no differences in outcomes between the two groups: 5-year event-free survival (EFS), 49% (95% confidence interval [CI]: 39-59) and 44% (95% CI: 30-58), and overall survival (OS), 51% (95% CI: 41-61) and 53.6% (95% CI: 40-68) in RMS2005 and ARST0531, respectively. In RMS2005, EFS of patients with FOXO1-positive tumors was significantly inferior to those with FOXO1-negative (49.3% vs 73%, P = .034). In contrast, in ARST0531, EFS of patients with FOXO1-positive tumors was 45% compared with 43.8% for those with FOXO1-negative.CONCLUSIONS: The outcome of patients with ARMS N1 was similar in both protocols. However, patients with FOXO1 fusion-negative tumors enrolled in RMS2005 showed a significantly better outcome, suggesting that different strategies of chemotherapy may have an impact in the outcome of this subgroup of patients.

AB - BACKGROUND: Treatment of children and adolescents with alveolar rhabdomyosarcoma (ARMS) and regional nodal involvement (N1) have been approached differently by North American and European cooperative groups. In order to define a better therapeutic strategy, we analyzed two studies conducted between 2005 and 2016 by the European paediatric Soft tissue sarcoma Study Group (EpSSG) and Children's Oncology Group (COG).METHODS: We retrospectively identified patients with ARMS N1 enrolled in either EpSSG RMS2005 or in COG ARST0531. Chemotherapy in RMS2005 comprised ifosfamide + vincristine + dactinomycin + doxorubicin (IVADo), IVA and maintenance (vinorelbine, cyclophosphamide); in ARST0531, it consisted of either vincristine + dactinomycin + cyclophosphamide (VAC) or VAC alternating with vincristine + irinotecan (VI). Local treatment was similar in both protocols.RESULTS: The analysis of the clinical characteristics of 239 patients showed some differences between study groups: in RMS2005, advanced Intergroup Rhabdomyosarcoma Study Group (IRS) and large tumors predominated. There were no differences in outcomes between the two groups: 5-year event-free survival (EFS), 49% (95% confidence interval [CI]: 39-59) and 44% (95% CI: 30-58), and overall survival (OS), 51% (95% CI: 41-61) and 53.6% (95% CI: 40-68) in RMS2005 and ARST0531, respectively. In RMS2005, EFS of patients with FOXO1-positive tumors was significantly inferior to those with FOXO1-negative (49.3% vs 73%, P = .034). In contrast, in ARST0531, EFS of patients with FOXO1-positive tumors was 45% compared with 43.8% for those with FOXO1-negative.CONCLUSIONS: The outcome of patients with ARMS N1 was similar in both protocols. However, patients with FOXO1 fusion-negative tumors enrolled in RMS2005 showed a significantly better outcome, suggesting that different strategies of chemotherapy may have an impact in the outcome of this subgroup of patients.

KW - Antineoplastic Combined Chemotherapy Protocols/therapeutic use

KW - Child

KW - Child, Preschool

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Infant

KW - Infant, Newborn

KW - Lymph Nodes/pathology

KW - Male

KW - Prognosis

KW - Retrospective Studies

KW - Rhabdomyosarcoma, Alveolar/drug therapy

KW - Survival Rate

UR - https://www.scopus.com/pages/publications/85096639971

U2 - 10.1002/pbc.28832

DO - 10.1002/pbc.28832

M3 - Article

C2 - 33245207

SN - 1545-5009

VL - 68

SP - e28832

JO - Pediatric blood & cancer

JF - Pediatric blood & cancer

IS - 3

M1 - e28832

ER -

Alveolar rhabdomyosarcoma with regional nodal involvement: Results of a combined analysis from two cooperative groups

Abstract

Keywords

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