Endometrial stromal sarcoma: A review of rare mesenchymal uterine neoplasm

Abstract

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.

New Aspects of Sarcomas of Uterine Corpus—A Brief Narrative Review

“…In 2014, the WHO officially reintroduced HGESS based on the identification of YWHAE-NUTM2A/B gene fusion (also known as YWHAE-FAM22A/B). The fusion of these two genes causes activation of 14-3-3 oncoprotein as a recurrent event in this more malignant subgroup of tumors [ 44 , 58 , 59 ]. Therefore, patients with HGESS experience earlier and more frequent recurrences (often <1 year) [ 57 ].…”

“…Therefore, patients with HGESS experience earlier and more frequent recurrences (often <1 year) [ 57 ]. The PFS (7–11 months) and OS (11–23 months) of HGESS are worse than those for LGESS [ 58 , 59 ]. YWHAE rearrangements have not been observed in other gynecological malignancies [ 47 ].…”

“…HGESS has a poor prognosis, as more than 50% of patients will present with an advanced stage of the disease. Lung metastases are most common [ 41 , 44 , 58 , 59 , 60 , 61 , 62 ].…”

“…UUS is an extremely rare and aggressive tumor with no specific line of differentiation that constitutes a diagnosis of exclusion [ 58 , 68 , 69 , 70 ]. The diagnosis should be performed after exclusion of other more common high-grade neoplasms (ULMS, carcinosarcoma, rhabdomyosarcoma, HGESS, LGESS, undifferentiated endometrial carcinoma and diffuse large B-cell lymphoma) ( Figure 8 ).…”

“…The diagnosis should be performed after exclusion of other more common high-grade neoplasms (ULMS, carcinosarcoma, rhabdomyosarcoma, HGESS, LGESS, undifferentiated endometrial carcinoma and diffuse large B-cell lymphoma) ( Figure 8 ). Approximately 60% of patients presented at advanced stage of the disease have a dismal prognosis (<2-year survival) [ 58 ]. UUS may arise in the endometrium or myometrium and is composed of pleomorphic or uniform cells with a high mitotic index that show no evidence of stromal or smooth muscle derivation [ 68 , 69 , 70 ].…”