Adult cervical embryonal rhabdomyosarcoma: a literature review of 41 adult cases with an illustrative aggressive case.

BACKGROUND: Adult cervical embryonal rhabdomyosarcoma (ERMS) is an exceptionally rare malignant mesenchymal tumor, and evidence regarding its clinicopathologic characteristics, optimal management, and prognostic factors remains limited. We report an aggressive case of adult cervical ERMS and provide a contemporary review of the literature. CASE PRESENTATION: A 50-year-old woman presented with abnormal vaginal bleeding and was diagnosed with cervical ERMS following histopathologic and immunohistochemical evaluation. She underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy followed by adjuvant vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy. Despite multimodal treatment, she developed rapid pelvic recurrence, progressive disease, and recurrent vaginal bleeding requiring palliative radiotherapy and died 13 months after diagnosis. To better characterize adult cervical ERMS, we reviewed English-language reports published between 2015 and 2026. Forty-one previously reported adult cases with sufficient clinical information were identified. The median age at diagnosis was 38 years, and the median tumor size was 5 cm. Most patients presented with stage I disease and were treated with surgery combined with chemotherapy, most commonly VAC-based regimens. Overall outcomes were generally favorable. Uncommon clinical presentations and associated conditions included uterine inversion, pregnancy-associated disease, cerebral venous sinus thrombosis, melanoma, and DICER1-associated alterations. CONCLUSION: Adult cervical ERMS demonstrates substantial clinical heterogeneity. Although most reported patients achieve favorable outcomes with multimodal treatment, a subset may experience aggressive disease characterized by rapid recurrence and poor prognosis. Greater molecular characterization, particularly regarding DICER1-associated alterations, may improve risk stratification and support more individualized treatment strategies.