Head and Neck Rhabdomyosarcoma in Children: a 20-Year Retrospective Study

Introduction:

The aim of this retrospective study was to assess rhabdomyosarcomas (RMS) of the head and neck in pediatric patients with regard to survival, treatment options, and clinical presentation.

Methods:

Data were retrospectively collected from patient charts with regard to head and neck RMS diagnosed at Charité – Universitätsmedizin Berlin between 1996 and 2016. Treatment modalities, clinical course, and side effects were analyzed. Survival analysis was carried out using the Kaplan-Meier method.

Results:

Twenty-eight patients (17 male, 11 female) with a mean age at diagnosis of 6.8+/-5.0 years have been included. Fourteen patients (50%) presented with painless swelling and nine patients (32.1%) presented with cranial nerve deficit at initial inspection. The location of the majority of rhabdomyosarcomas was the orbit (n = 9; 32.1%), followed by parapharyngeal (n = 4; 14.2%) and sinonasal site (n = 3; 10.7%). All patients (n = 28; 100%) received polychemotherapy after histological confirmation. Additional adjuvant radiation therapy was carried out in 24 patients (85.7%), and surgery was performed in 12 cases (42.9%). The 5-year overall survival was 91.3%, and median progression-free survival was 46+/-67.4 months. Common side effects resulting from chemotherapy and irradiation included mucositis, neutropenia, nausea and vomiting, and radiodermatitis, long-term side effects presented as facial mutilation and microsomia.

Conclusions:

In pediatric patients multimodality treatment of head and neck rhabdomyosarcomas can lead to an excellent 5-year overall survival of up to 91.3%.

Publication History

Publication Date:
18 April 2018 (online)