Embryonal rhabdomyosarcoma of the female gynecologic tract: a retrospective clinical study of 14 cases

Abstract

Background The purposes of this study were to investigate the clinical features, treatments, and prognosis of embryonal rhabdomyosarcomas (ERMS) in the female genital tract with long-term follow-up. Methods The data of 14 patients who diagnosis of ERMS in the genitourinary system from January 2010 to October 2022 were retrospectively screened. The roles of prognostic risk factors, such as age, tumor size, tumor grade, depth of myometrial infiltration, lymphvascular space invasion and primary treatment were evaluated. Results We report 14 ERMS that arose in patients aged 10 to 44 (median = 19) year. The median longest tumor diameter was 5.5 cm (range, 1.0–15.0 cm). There was no myometrial infiltration in 4 patients, superficial myometrial infiltration in 3 patients, deep myometrial infiltration in 5 patients. Lymphvascular space invasion (LVSI) was present in 2 patients, and absent in 10 patients. All of the patients performed surgery and chemotherapy, and one patient also received radiotherapy. Follow-up evaluation was lost in two patients after three or six months of combination therapy. The 2-year PFS was 83.3% and the 5-year DSS was 91.7% with a median follow-up time of 57 months (range, 10–132). Patients with LVSI (+) had a significantly increased time to progression compared with patients with LVSI (-) (PFS: p = 0.004; DSS: p = 0.046). There was a trend toward improved PFS and DSS with early-stage disease (IRSG group I/FIGO stage I (uterine sarcoma or adenosarcoma)/T1) compared to advanced-stage disease. Conclusions ERMS in the female gynecologic tract with early stage has a good prognosis, a conservative surgical approach combined with chemotherapy can lead to good outcomes, preserving-fertility may be possible for younger women of these patients.