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Head Neck. 2017 Jan;39(1):24-31. doi: 10.1002/hed.24547. Epub 2016 Jul 26.

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

Author information

1
Adolescent and Pediatric Oncology Department, Institut Curie, Paris, France.
2
Department of Biostatistics, Institut Curie, Paris, France.
3
Paediatric Oncology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
4
Pathology Department, Royal Manchester Children's Hospital, Manchester, United Kingdom.
5
Pediatric Haematology Oncology Department, Hopital Universitaire des enfants, Brussels, Belgium.
6
Department of Paediatric Oncology, Royal Manchester Children's Hospital, Manchester, United Kingdom.
7
Center of Pediatric Oncology, Princess Máxima, Utrecht, The Netherlands.
8
Department of Pediatric Oncology, Institut d'Hematologie et d'Oncologie Pédiatrique, Lyon, France.
9
Department of Pediatric Oncology, Emma Children's Hospital - Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
10
Department of Paediatrics, Rigshospitalet, Copenhagen, Denmark.
11
Department of Pediatric Oncology, Children's Hospital for Wales, Heath Park, Cardiff, United Kingdom.
12
Pediatric Oncology Unit, Gustave-Roussy, Villejuif, France.
13
School of Clinical Sciences, University of Bristol, Bristol, United Kingdom.

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.

KEYWORDS:

International Society of Pediatric Oncology Malignant Mesenchymal Tumor (SIOP-MMT) protocols; children; head and neck rhabdomyosarcoma; radiotherapy; surgery

PMID:
27459057
DOI:
10.1002/hed.24547
[Indexed for MEDLINE]

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