Details

Autor(en) / Beteiligte

• 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

Titel

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

Ist Teil von

• Head & neck, 2017-01, Vol.39 (1), p.24-31

Ort / Verlag

United States: Wiley Subscription Services, Inc

Erscheinungsjahr

2017

Quelle

Wiley Online Library Journals Frontfile Complete

Beschreibungen/Notizen

• 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