Advanced Orofacial Rhabdomyosarcoma: A Retrospective Study of 31 Cases

Abstract

Introduction Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma encountered in childhood and adolescence. Early diagnosis of pediatric cases is critical to improving outcomes, especially when socioeconomic status and geographical access to specialist services can reduce opportunities for early cancer detection and treatment.Objective The objective of this study is to determine factors that can delay referral and treatment in specialist pediatric oncology center upon our population specificities.Methods This retrospective study involved 31 children between 2003 and 2013. Children affected by histologically confirmed RMS occurring as a primary lesion in the orofacial area were included.Results The median age was 8 ± 4.22 years (range: 3 months – 15 years). The male to female ratio was 1.8:1. Most of the patients had advanced stage disease at presentation (81.7% group had 3–4 pretreatment staging) with parameningeal involvement in 80.6% of the cases. The 2-year event-free survival rate was 17.7 ± 7.8% for all the patients. Delay of admission to our unit and abandonment of treatment seem to be important factors for the dismal prognosis.Conclusion Patient's location, socioeconomic status and health care coverage have had an impact on longer delays in seeking care and on follow-up. More studies are needed for implementation of a better management practices and a better supportive care upon specificities of our population.

Simultaneous diagnosis and resection of orofacial rhabdomyosarcoma with frozen section biopsy: a case report

“…Anatomically, rhabdomyosarcoma of the head and neck can be categorized into two groups: parameningeal rhabdomyosarcoma including the nose, nasopharynx, paranasal sinus and middle ear and non-parameningeal rhabdomyosarcoma including the scalp, orbit, oral cavity and oropharynx [ 4 ]. Additionally, there are two major histologic subtypes of rhabdomyosarcoma: embryonal rhabdomyosarcoma, which typically arises in head and neck or genitourinary sites, and alveolar rhabdomyosarcoma, which occurs in the trunk and extremities [ 5 ].…”

“…Rhabdomyosarcoma is an aggressive skeletal muscle malignancy of mesenchymal origin and is classified as a small round cell tumor [ 3 ]. Although the head and neck are the most common sites of rhabdomyosarcoma (35%), oral lesions are relatively uncommon and account for only 10% to 12% of head and neck rhabdomyosarcoma cases [ 4 ]. While many factors, including patient age and the location, size and resectability of the primary tumor, must be considered in order to select the proper treatment option for rhabdomyosarcoma, primary resection or at least open incisional biopsy is one of the key aspects of managing rhabdomyosarcoma [ 1 ].…”

Clinical Significance of Transnasal Endoscopic Surgery in the Treatment of Children With Localized Parameningeal Sarcomas

“…По лока-лизации саркомы головы и шеи разделяют на два анатомических региона -пара-и непараменин-геальный, различающиеся прогнозом заболева-ния. К параменингеальной локализации относятся опухоли, расположенные в полости носа, носоглот-ке, околоносовых пазухах, среднем ухе, височной кости, а также в подвисочной и крылонебной ямках [1]. Другие морфологические варианты сарком мягких тканей параменингеальной локализации и саркомы костей основания черепа составляют не более 1% среди всех злокачественных опухолей детского возраста.…”