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

*Corresponding author for this work

Research output: Contribution to journalArticleResearchpeer-review

12 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.

Original languageAmerican English
Pages (from-to)24-31
Number of pages8
JournalHead and Neck
Volume39
Issue number1
DOIs
Publication statusPublished - 1 Jan 2017

Keywords

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

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