Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging: Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies

Timothy Rogers; Veronique Minard-Colin; Nathalie Cozic; Meriel Jenney; Johannes H M Merks; Soledad Gallego; Christine Devalck; Mark N Gaze; Anna Kelsey; Odile Oberlin; Mike Stevens; Richard D Spicer; Christophe Bergeron; Helene Martelli

doi:10.1002/pbc.26486

Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging: Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies

Timothy Rogers
, Veronique Minard-Colin
, Nathalie Cozic
, Meriel Jenney
, Johannes H M Merks
, Soledad Gallego
, Christine Devalck
, Mark N Gaze
, Anna Kelsey
, Odile Oberlin
, Mike Stevens
, Richard D Spicer
, Christophe Bergeron
, Helene Martelli

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

26 Citations (Scopus)

Abstract

PURPOSE: To report the results from International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors studies (MMT 89 and 95) of males with nonmetastatic paratesticular rhabdomyosarcoma.

METHODS: From 1989 to 2003, 159 patients were included. Radical inguinal orchidectomy was recommended, but retroperitoneal lymph node (LN) assessment was based on imaging alone. The treatment was stratified by stage (SIOP tumor-node-metastasis staging system) and histology.

RESULTS: Median age at presentation was 5.6 years (range 0.3-17.6) and 120 patients were of <10 years (75%). Patients ≥10 years had tumors of >5 cm more frequently compared to patients of <10 years (54% vs. 22%, P = 0.0004). The 5- year overall and progression-free survivals were 94% and 83%, respectively. Seventy-eight percent of relapses occurred in the retroperitoneal LN. Thirty-one percent of stage N0 patients of age ≥10 years developed node relapse, compared with 8% of N0 patients aged <10 years (P = 0.0005).

CONCLUSIONS: Older patients with paratesticular rhabdomyosarcoma have a significant risk of LN relapse. These results support a surgical approach to LN staging in this subgroup of patients.

Original language	English
Article number	e26486
Journal	Pediatric blood & cancer
Volume	64
Issue number	9
DOIs	https://doi.org/10.1002/pbc.26486
Publication status	Published - Feb 2017
Externally published	Yes

Keywords

Adolescent
Antineoplastic Combined Chemotherapy Protocols/therapeutic use
Child
Child, Preschool
Combined Modality Therapy
Disease-Free Survival
Humans
Infant
Kaplan-Meier Estimate
Lymphatic Metastasis/diagnosis
Male
Neoplasm Staging/methods
Orchiectomy/methods
Retroperitoneal Neoplasms/mortality
Rhabdomyosarcoma/mortality
Scrotum/surgery
Young Adult

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Rogers, T., Minard-Colin, V., Cozic, N., Jenney, M., Merks, J. H. M., Gallego, S., Devalck, C., Gaze, M. N., Kelsey, A., Oberlin, O., Stevens, M., Spicer, R. D., Bergeron, C., & Martelli, H. (2017). Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging: Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies. Pediatric blood & cancer, 64(9), Article e26486. https://doi.org/10.1002/pbc.26486

Rogers, Timothy ; Minard-Colin, Veronique ; Cozic, Nathalie et al. / Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging : Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies. In: Pediatric blood & cancer. 2017 ; Vol. 64, No. 9.

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title = "Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging: Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies",

abstract = "PURPOSE: To report the results from International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors studies (MMT 89 and 95) of males with nonmetastatic paratesticular rhabdomyosarcoma.METHODS: From 1989 to 2003, 159 patients were included. Radical inguinal orchidectomy was recommended, but retroperitoneal lymph node (LN) assessment was based on imaging alone. The treatment was stratified by stage (SIOP tumor-node-metastasis staging system) and histology.RESULTS: Median age at presentation was 5.6 years (range 0.3-17.6) and 120 patients were of <10 years (75\%). Patients ≥10 years had tumors of >5 cm more frequently compared to patients of <10 years (54\% vs. 22\%, P = 0.0004). The 5- year overall and progression-free survivals were 94\% and 83\%, respectively. Seventy-eight percent of relapses occurred in the retroperitoneal LN. Thirty-one percent of stage N0 patients of age ≥10 years developed node relapse, compared with 8\% of N0 patients aged <10 years (P = 0.0005).CONCLUSIONS: Older patients with paratesticular rhabdomyosarcoma have a significant risk of LN relapse. These results support a surgical approach to LN staging in this subgroup of patients.",

keywords = "Adolescent, Antineoplastic Combined Chemotherapy Protocols/therapeutic use, Child, Child, Preschool, Combined Modality Therapy, Disease-Free Survival, Humans, Infant, Kaplan-Meier Estimate, Lymphatic Metastasis/diagnosis, Male, Neoplasm Staging/methods, Orchiectomy/methods, Retroperitoneal Neoplasms/mortality, Rhabdomyosarcoma/mortality, Scrotum/surgery, Young Adult",

author = "Timothy Rogers and Veronique Minard-Colin and Nathalie Cozic and Meriel Jenney and Merks, \{Johannes H M\} and Soledad Gallego and Christine Devalck and Gaze, \{Mark N\} and Anna Kelsey and Odile Oberlin and Mike Stevens and Spicer, \{Richard D\} and Christophe Bergeron and Helene Martelli",

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Rogers, T, Minard-Colin, V, Cozic, N, Jenney, M, Merks, JHM, Gallego, S, Devalck, C, Gaze, MN, Kelsey, A, Oberlin, O, Stevens, M, Spicer, RD, Bergeron, C & Martelli, H 2017, 'Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging: Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies', Pediatric blood & cancer, vol. 64, no. 9, e26486. https://doi.org/10.1002/pbc.26486

Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging: Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies. / Rogers, Timothy; Minard-Colin, Veronique; Cozic, Nathalie et al.
In: Pediatric blood & cancer, Vol. 64, No. 9, e26486, 02.2017.

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

TY - JOUR

T1 - Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging

T2 - Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies

AU - Rogers, Timothy

AU - Minard-Colin, Veronique

AU - Cozic, Nathalie

AU - Jenney, Meriel

AU - Merks, Johannes H M

AU - Gallego, Soledad

AU - Devalck, Christine

AU - Gaze, Mark N

AU - Kelsey, Anna

AU - Oberlin, Odile

AU - Stevens, Mike

AU - Spicer, Richard D

AU - Bergeron, Christophe

AU - Martelli, Helene

PY - 2017/2

Y1 - 2017/2

N2 - PURPOSE: To report the results from International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors studies (MMT 89 and 95) of males with nonmetastatic paratesticular rhabdomyosarcoma.METHODS: From 1989 to 2003, 159 patients were included. Radical inguinal orchidectomy was recommended, but retroperitoneal lymph node (LN) assessment was based on imaging alone. The treatment was stratified by stage (SIOP tumor-node-metastasis staging system) and histology.RESULTS: Median age at presentation was 5.6 years (range 0.3-17.6) and 120 patients were of <10 years (75%). Patients ≥10 years had tumors of >5 cm more frequently compared to patients of <10 years (54% vs. 22%, P = 0.0004). The 5- year overall and progression-free survivals were 94% and 83%, respectively. Seventy-eight percent of relapses occurred in the retroperitoneal LN. Thirty-one percent of stage N0 patients of age ≥10 years developed node relapse, compared with 8% of N0 patients aged <10 years (P = 0.0005).CONCLUSIONS: Older patients with paratesticular rhabdomyosarcoma have a significant risk of LN relapse. These results support a surgical approach to LN staging in this subgroup of patients.

AB - PURPOSE: To report the results from International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors studies (MMT 89 and 95) of males with nonmetastatic paratesticular rhabdomyosarcoma.METHODS: From 1989 to 2003, 159 patients were included. Radical inguinal orchidectomy was recommended, but retroperitoneal lymph node (LN) assessment was based on imaging alone. The treatment was stratified by stage (SIOP tumor-node-metastasis staging system) and histology.RESULTS: Median age at presentation was 5.6 years (range 0.3-17.6) and 120 patients were of <10 years (75%). Patients ≥10 years had tumors of >5 cm more frequently compared to patients of <10 years (54% vs. 22%, P = 0.0004). The 5- year overall and progression-free survivals were 94% and 83%, respectively. Seventy-eight percent of relapses occurred in the retroperitoneal LN. Thirty-one percent of stage N0 patients of age ≥10 years developed node relapse, compared with 8% of N0 patients aged <10 years (P = 0.0005).CONCLUSIONS: Older patients with paratesticular rhabdomyosarcoma have a significant risk of LN relapse. These results support a surgical approach to LN staging in this subgroup of patients.

KW - Adolescent

KW - Antineoplastic Combined Chemotherapy Protocols/therapeutic use

KW - Child

KW - Child, Preschool

KW - Combined Modality Therapy

KW - Disease-Free Survival

KW - Humans

KW - Infant

KW - Kaplan-Meier Estimate

KW - Lymphatic Metastasis/diagnosis

KW - Male

KW - Neoplasm Staging/methods

KW - Orchiectomy/methods

KW - Retroperitoneal Neoplasms/mortality

KW - Rhabdomyosarcoma/mortality

KW - Scrotum/surgery

KW - Young Adult

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DO - 10.1002/pbc.26486

M3 - Article

C2 - 28205365

SN - 1545-5009

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JO - Pediatric blood & cancer

JF - Pediatric blood & cancer

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Rogers T, Minard-Colin V, Cozic N, Jenney M, Merks JHM, Gallego S et al. Paratesticular rhabdomyosarcoma in children and adolescents-Outcome and patterns of relapse when utilizing a nonsurgical strategy for lymph node staging: Report from the International Society of Paediatric Oncology (SIOP) Malignant Mesenchymal Tumour 89 and 95 studies. Pediatric blood & cancer. 2017 Feb;64(9):e26486. doi: 10.1002/pbc.26486