Nonparameningeal head and neck rhabdomyosarcoma in children and adolescents: Lessons from the consecutive International Society of Pediatric Oncology Malignant Mesenchymal Tumor studies

Daniel Orbach; Veronique Mosseri; Soledad Gallego; Anna Kelsey; Christine Devalck; Bernadette Brenann; Max M van Noesel; Christophe Bergeron; Johannes H M Merks; Catherine Rechnitzer; Meriel Jenney; Veronique Minard-Colin; Michael Stevens

doi:10.1002/hed.24547

Nonparameningeal head and neck rhabdomyosarcoma in children and adolescents: Lessons from the consecutive International Society of Pediatric Oncology Malignant Mesenchymal Tumor studies

Daniel Orbach, Veronique Mosseri, Soledad Gallego, Anna Kelsey, Christine Devalck, Bernadette Brenann, Max M van Noesel, Christophe Bergeron, Johannes H M Merks, Catherine Rechnitzer, Meriel Jenney, Veronique Minard-Colin, Michael Stevens

Department of Paediatrics and Adolescent Medicine

25 Citations (Scopus)

Abstract

BACKGROUND: This article reports risk factors and long-term outcome in localized nonparameningeal head and neck rhabdomyosarcomas in children and adolescents from a combined dataset from 3 consecutive international trials.

METHODS: Data from 140 children (9.3% of total) prospectively enrolled in the International Society of Pediatric Oncology Malignant Mesenchymal Tumor (SIOP-MMT)-84/89/95 studies were analyzed.

RESULTS: Primary site was: superficial face in 46%; oral cavity (21%); neck (19%); and salivary glands (14%). Local control was achieved in 96%, but 49% relapsed (locoregionally 91%). At median follow-up of 10 years, 5-year overall survival (OS) was 74.7% (67.4% to 81.9%) and event-free survival 48.9% (40.6% to 57.2%), although this improved with successive studies. Radiotherapy (RT) as first-line treatment was independently prognostic for event-free survival (relative risk [RR] = 0.4 [range, 0.2-0.7]; p < .01) even if it did not impact OS (RR = 1 [range, 0.5-2]).

CONCLUSION: High rates of locoregional relapse were seen in head and neck rhabdomyosarcoma that should be prevented by more frequent use of RT in this primary. © 2016 Wiley Periodicals, Inc. Head Neck 39: 24-31, 2017.

Original language	English
Journal	Head and Neck
Volume	39
Issue number	1
Pages (from-to)	24-31
Number of pages	8
ISSN	1043-3074
DOIs	https://doi.org/10.1002/hed.24547
Publication status	Published - Jan 2017

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Orbach, D., Mosseri, V., Gallego, S., Kelsey, A., Devalck, C., Brenann, B., van Noesel, M. M., Bergeron, C., Merks, J. H. M., Rechnitzer, C., Jenney, M., Minard-Colin, V., & Stevens, M. (2017). Nonparameningeal head and neck rhabdomyosarcoma in children and adolescents: Lessons from the consecutive International Society of Pediatric Oncology Malignant Mesenchymal Tumor studies. Head and Neck, 39(1), 24-31. https://doi.org/10.1002/hed.24547

Nonparameningeal head and neck rhabdomyosarcoma in children and adolescents: Lessons from the consecutive International Society of Pediatric Oncology Malignant Mesenchymal Tumor studies. / Orbach, Daniel; Mosseri, Veronique; Gallego, Soledad et al.
In: Head and Neck, Vol. 39, No. 1, 01.2017, p. 24-31.

Research output: Contribution to journal › Journal article › Research › peer-review

Orbach, D, Mosseri, V, Gallego, S, Kelsey, A, Devalck, C, Brenann, B, van Noesel, MM, Bergeron, C, Merks, JHM, Rechnitzer, C, Jenney, M, Minard-Colin, V & Stevens, M 2017, 'Nonparameningeal head and neck rhabdomyosarcoma in children and adolescents: Lessons from the consecutive International Society of Pediatric Oncology Malignant Mesenchymal Tumor studies', Head and Neck, vol. 39, no. 1, pp. 24-31. https://doi.org/10.1002/hed.24547

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abstract = "BACKGROUND: This article reports risk factors and long-term outcome in localized nonparameningeal head and neck rhabdomyosarcomas in children and adolescents from a combined dataset from 3 consecutive international trials.METHODS: Data from 140 children (9.3% of total) prospectively enrolled in the International Society of Pediatric Oncology Malignant Mesenchymal Tumor (SIOP-MMT)-84/89/95 studies were analyzed.RESULTS: Primary site was: superficial face in 46%; oral cavity (21%); neck (19%); and salivary glands (14%). Local control was achieved in 96%, but 49% relapsed (locoregionally 91%). At median follow-up of 10 years, 5-year overall survival (OS) was 74.7% (67.4% to 81.9%) and event-free survival 48.9% (40.6% to 57.2%), although this improved with successive studies. Radiotherapy (RT) as first-line treatment was independently prognostic for event-free survival (relative risk [RR] = 0.4 [range, 0.2-0.7]; p < .01) even if it did not impact OS (RR = 1 [range, 0.5-2]).CONCLUSION: High rates of locoregional relapse were seen in head and neck rhabdomyosarcoma that should be prevented by more frequent use of RT in this primary. {\textcopyright} 2016 Wiley Periodicals, Inc. Head Neck 39: 24-31, 2017.",

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AU - Gallego, Soledad

AU - Kelsey, Anna

AU - Devalck, Christine

AU - Brenann, Bernadette

AU - van Noesel, Max M

AU - Bergeron, Christophe

AU - Merks, Johannes H M

AU - Rechnitzer, Catherine

AU - Jenney, Meriel

AU - Minard-Colin, Veronique

AU - Stevens, Michael

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N2 - BACKGROUND: This article reports risk factors and long-term outcome in localized nonparameningeal head and neck rhabdomyosarcomas in children and adolescents from a combined dataset from 3 consecutive international trials.METHODS: Data from 140 children (9.3% of total) prospectively enrolled in the International Society of Pediatric Oncology Malignant Mesenchymal Tumor (SIOP-MMT)-84/89/95 studies were analyzed.RESULTS: Primary site was: superficial face in 46%; oral cavity (21%); neck (19%); and salivary glands (14%). Local control was achieved in 96%, but 49% relapsed (locoregionally 91%). At median follow-up of 10 years, 5-year overall survival (OS) was 74.7% (67.4% to 81.9%) and event-free survival 48.9% (40.6% to 57.2%), although this improved with successive studies. Radiotherapy (RT) as first-line treatment was independently prognostic for event-free survival (relative risk [RR] = 0.4 [range, 0.2-0.7]; p < .01) even if it did not impact OS (RR = 1 [range, 0.5-2]).CONCLUSION: High rates of locoregional relapse were seen in head and neck rhabdomyosarcoma that should be prevented by more frequent use of RT in this primary. © 2016 Wiley Periodicals, Inc. Head Neck 39: 24-31, 2017.

AB - BACKGROUND: This article reports risk factors and long-term outcome in localized nonparameningeal head and neck rhabdomyosarcomas in children and adolescents from a combined dataset from 3 consecutive international trials.METHODS: Data from 140 children (9.3% of total) prospectively enrolled in the International Society of Pediatric Oncology Malignant Mesenchymal Tumor (SIOP-MMT)-84/89/95 studies were analyzed.RESULTS: Primary site was: superficial face in 46%; oral cavity (21%); neck (19%); and salivary glands (14%). Local control was achieved in 96%, but 49% relapsed (locoregionally 91%). At median follow-up of 10 years, 5-year overall survival (OS) was 74.7% (67.4% to 81.9%) and event-free survival 48.9% (40.6% to 57.2%), although this improved with successive studies. Radiotherapy (RT) as first-line treatment was independently prognostic for event-free survival (relative risk [RR] = 0.4 [range, 0.2-0.7]; p < .01) even if it did not impact OS (RR = 1 [range, 0.5-2]).CONCLUSION: High rates of locoregional relapse were seen in head and neck rhabdomyosarcoma that should be prevented by more frequent use of RT in this primary. © 2016 Wiley Periodicals, Inc. Head Neck 39: 24-31, 2017.

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Nonparameningeal head and neck rhabdomyosarcoma in children and adolescents: Lessons from the consecutive International Society of Pediatric Oncology Malignant Mesenchymal Tumor studies

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