Pleomorphic Rhabdomyosarcoma of the Uterus - Case Report and a Systematic Review of the Literature.

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.

Systematic pelvic and paraaortic lymphadenectomy in early high-risk or advanced endometrial cancer.

PURPOSE: Patients with high-risk early or advanced endometrial cancer (EC) are at high risk for lymph node (LN) metastases. However, both the anatomical pattern of the LN metastases and also the therapeutic value of systematic LN dissection remain a field of discussion and controversy. METHODS: We performed an exploratory analysis of patients with high-risk or advanced EC who underwent systematic pelvic and para-aortic lymphadenectomy in two tertiary referral centers for gynecological malignancies. RESULTS: One hundred and twenty-eight completely surgically staged patients underwent systematic pelvic and para-aortic lymphadenectomy for high-risk or advanced EC. A median of 29 and 21.5 LN was harvested in the pelvis and in the para-aortic region, respectively. Overall, 27 patients (21.1 %) had positive LN: 18 % showed positive pelvic LN and 14.8 % positive para-aortic LN; while 3.1 % showed isolated para-aortic LN metastases. Five-year overall survival was 70 versus 30 % in LN-negative versus LN-positive patients (p < 0.01). LN-status was the only factor significantly associated with overall survival [HR: 3.67 (95 % CI 1.48-9.11); p = 0.01] in a multivariate Cox regression model. CONCLUSIONS: Patients with high-risk or advanced EC were at a high-risk for LN metastases. Anatomical distribution of positive LN indicates that lymphadenectomy, when performed, should contain both pelvic and para-aortic areas up to the renal vessels for an accurate assessment of all potential positive LN.