Objective: This review aims to analyze the pathological aspects, diagnosis and treatment of rare mesenchymal uterine tumors. Methods: On August 2019, a systematic review of the literature was done on Pubmed, MEDLINE, Scopus, and Google Scholar search engines. The systematic review was carried out in agreement with the Preferred Reporting Items for Systematic Reviews and Meta-Analyzes statement (PRISMA). The following words and key phrases have been searched: "endometrial stromal sarcoma", "low-grade endometrial stromal sarcoma", "high-grade endometrial stromal sarcoma", "uterine sarcoma", "mesenchymal uterine tumors" and "uterine stromal sarcoma". Across these platforms and research studies, five main aspects were analyzed: the biological characteristics of the neoplasms, the number of cases, the different therapeutic approaches used, the follow-up and the oncological outcomes. Results: Of the 94 studies initially identified, 55 were chosen selecting articles focusing on endometrial stromal sarcoma. Of these fifty-five studies, 46 were retrospective in design, 7 were reviews and 2 randomized phases III trials. Conclusion: Endometrial stromal sarcomas are rare mesenchymal uterine neoplasms and surgery represents the standard treatment. For uterus-limited disease, the remove en bloc with an intact resection of the tumor (without the use of morcellation) is strongly recommended. For advanced-stage disease, the standard surgical treatment is adequate cytoreduction with metastatectomy. Pelvic and para-aortic lymphadenectomy is not recommended in patients with Low-grade Endometrial Stromal Sarcoma (ESS), while is not clear whether cytoreduction of advanced tumors improves patient survival in High-grade ESS. Administration of adjuvant radiotherapy or chemotherapy is not routinely used and its role is still debated.

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was not associated with DFS (p=0.518) (Hazard ratio (HR) 1.20 95% CI 0.688-2.100). We found differences according to the surgical approach for OS (p<0.043) with an HR of 1.63 (95% CI: 1.104-1.63). There is 1.63 times more risk of death in the laparotomy approach. 38 (49.35%) of the laparotomies were performed in the first 5 years of the series, patients died from other causes not due to cancer (18 (60%) versus 22 (37.9%) in the laparoscopy group p=0.049). We have compared OS with cancer-specific death in both groups, this difference was not significant (p=0.673) (HR 1.15 95% CI 0.587-2.28). We have not found differences in local and distant recurrence (p=0.491), or recurrence above vaginal vault (p=0.534) in both groups. Conclusion Surgical approach had no impact on DFS or OS in our series. Corresponding to the first years of the series, OS is lower in the LPM group, but when analyzing OS with cancer-specific death, this difference was not significant.

<b><i>Objective:</i></b> Atypical endometrial hyperplasia (AH) is the neoplastic precursor more often associated with endometrial cancer (EC). Nowadays, 25–50% of patients subjected to hysterectomy for preoperative AH are diagnosed with EC at the final pathological analysis. Furthermore, there is no consensus on which preoperative AH patients would benefit from sentinel lymph node mapping. This study aimed to evaluate nodal assessment and preoperative cancer risk factors in preoperative AH patients undergoing nodal surgical staging. <b><i>Methods:</i></b> Patients undergoing surgical treatment for AH were retrospectively included in the analysis. Patients were divided into two groups (AH and EC groups) based on the final surgical pathology. The ESGO/ESTRO/ESP risk classification was used for EC cases. <b><i>Design:</i></b> This was a retrospective study. <b><i>Results:</i></b> Of the 207 AH patients treated, 152 cases met the inclusion criteria. Among preoperative AH patients with final EC diagnosis, 39 patients were in the low-risk group (25.7%), 8 in the intermediate-risk group (5.3%), 4 in high-intermediate (2.6%), and 3 patients were allocated in the high-risk group (2.0%). Fifty-four total patients underwent nodal surgical staging. Only one nodal micrometastasis (0.7%) was found at ultrastaging. Multivariate analysis showed abnormal uterine bleeding (AUB) (<i>p</i> = 0.01), hypertension (<i>p</i> &#x3c; 0.01), and endometrial thickness ≥20 mm (<i>p</i> = 0.02) statistically more represented in patients with EC at final surgical analysis. EC risk was 2.9 (95% CI: 1.29–6.48) in AUB, 2.7 (95% CI: 1.06–6.92) in hypertension, and 3.1 (95% CI: 1.19–7.97) in endometrial thickness ≥20 mm cases. <b><i>Limitations:</i></b> The present study has limitations inherent in its retrospective nature. <b><i>Conclusion:</i></b> The overall risk of nodal metastases in preoperative AH patients was low. Conversely, 9.9% of the preoperative AH patients belonged to the intermediate or high-risk group for EC at the final histological examination. Preoperative cancer risk factors would identify AH patients for whom nodal staging could be suggested.