Pleomorphic rhabdomyosarcoma of the uterus - Case report and a systematic review of the literature

Abstract

Background: Pleomorphic rhabdomyosarcomas of the uterus, mainly occurring in postmenopausal women with leading symptoms of vaginal bleeding and abdominal pain, are very rare malignant tumors of the female genital tract. Due to the inefficiency of the adjuvant therapy, the outcome remains poor in the majority of the reported cases. Patient and Methods: We present a case of a 73-year-old patient diagnosed with pleomorphic rhabdomyosarcoma of the uterus. Together with the case report, a systematic review of the literature is presented focusing on different treatment strategies and their outcome. The 95% confidence interval (CI) of the overall mean survival and the respective mean survival of each different treatment strategy was calculated using SAS Studio. Results: In the presented case, the patient showed no symptoms and was admitted into hospital due to abnormal uterine findings during a routine gynecological examination. Vaginal ultrasound scans showed a severely enlarged and intracavitaryly filled uterus. The patient underwent hysterectomy, as well as bilateral salpingo-oophorectomy. Regarding the systematic review of the literature, patients with adjuvant chemotherapy show the best outcome with a mean survival rate of 15.8±7.3 months (one patient excluded), whereas with a mean survival rate of 4.1±5.2 months, patients with sole surgical treatment show the shortest survival after diagnosis. Conclusion: Although there is no standardized approach in the treatment of this rare disease, we present a differentiated overview.