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

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

来源：Head and Neck-Journal for the Sciences and Specialties of the Head and Neck, 2017, 39(1): 24-31.

DOI：10.1002/hed.24547

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

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 improvedwith successive studies. Radiotherapy (RT) as first-line treatment was independently prognostic for event-free survival (relative risk [RR] 50.4 [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.

• 出版日期2017-1