Clinical Features and Treatment Results in Children With Head and Neck Rhabdomyosarcoma

Rhabdomyosarcoma constitutes 3% to 4% of childhood cancers, with nearly half seen in the head and neck location. We aimed to investigate the clinical features and treatment outcomes of 65 children diagnosed and treated for head and neck rhabdomyosarcoma (RMS) between 2004 and 2018. The median age was 5.8 years with a 37:28 M/F ratio. The primary location was parameningeal in 49.2%, orbital in 35.4%, and other nonparameningeal in 15.4% patients. The most common histopathologic subtype was the embryonal subtype (73.8%). The chemotherapy regimens of CDCV (cisplatin, doxorubicin, cyclophosphamide, vincristine); VAC/VAdrC (vincristine, actinomycin-D, cyclophosphamide/vincristine, doxorubicin, cyclophosphamide); PIAV (ifosfamide, cisplatin, vincristine, doxorubicin); and VDC/IE (vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide) were used depending on the years of diagnosis. The tumor location, risk grouping, and stage were found as the significant prognostic factors. The 5-year event-free survival (EFS) rate for all patients 41.2% and the overall survival (OS) rate was 59.3%. The 5-year OS rates were 85.2% and 80% in the orbital and other nonparameningeal RMS, respectively, it was 34.2% in the parameningeal RMS patients ( P =0.01). The patients with advanced stage, parameningeal disease have poor prognosis. New treatment approaches should be investigated to improve the outcomes in these groups.