Clinical characteristics and prognosis of rhabdomyosarcoma in the female reproductive system: a population-based analysis using SEER data.

Abstract

Objective: Rhabdomyosarcoma of the female reproductive system is rare, and as a result, management strategies are unclear. In this study, we categorized rhabdomyosarcoma based on location (cervical, uterine, vulvovaginal) and analyzed prognostic factors to guide individualized treatment.

Methods: Data from the Surveillance, Epidemiology, and End Results database (2000-2021) were used to identify patients with cervical, uterine, and vulvovaginal rhabdomyosarcoma. Multivariate Cox regression identified prognostic factors, and Kaplan-Meier analysis assessed overall survival and disease-specific survival.

Results: For cervical rhabdomyosarcoma (n = 76), radical surgery improved disease-specific survival (91.9% vs 62.8%, p = .005), but conservative surgery was sufficient for patients aged <49 years, with a tumor diameter <4 cm, or with the embryonal subtype. In uterine rhabdomyosarcoma (n = 253), the embryonal subtype showed better overall survival (62.3% vs 23%, p < .0001) and disease-specific survival (65.9% vs 31%, p < .0001), especially for tumors ≤14 cm. For vulvovaginal rhabdomyosarcoma (n = 60), no survival differences were observed between local and radical surgery in patients aged <16 years or with tumors <8 cm. Adjuvant chemotherapy in cervical rhabdomyosarcoma has been shown to enhance the overall survival rate of patients undergoing radical surgery; however, radiotherapy appears to diminish their overall survival outcomes. In the case of uterine rhabdomyosarcoma of the embryonic subtype, chemotherapy concurrently improves both overall and disease-specific survival rates, while radiotherapy does not demonstrate a significant impact. For vulvovaginal rhabdomyosarcoma, chemotherapy is effective in improving the overall survival rate among non-surgical patients, whereas radiotherapy shows no effect on the survival outcomes across all subgroups.

Conclusions: Conservative treatment is suitable for young patients with cervical rhabdomyosarcoma who have small tumors or the embryonal subtype, while radical surgery benefits the overall population. Embryonal uterine rhabdomyosarcoma has better outcomes, and young patients with vulvovaginal rhabdomyosarcoma of the embryonal subtype can undergo local surgery. For these 3 sites, chemotherapy is indispensable, while radiotherapy should be administered with caution.