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

Abstract

Sarcomas of the uterine corpus are rare malignant neoplasms, which are further classified into mesenchymal tumors, and mixed (epithelial plus mesenchymal) tumors. The main issues concerning these neoplasms are the small number of clinical trials, insufficient data from evidence-based medicine, insignificant interest from the pharmaceutical industry, all of which close a vicious circle. The low frequency of these malignancies implies insufficient experience in the diagnosis, hence incomplete surgical and complex treatment. Additionally, the rarity of these sarcomas makes it very difficult to develop clinical practice guidelines. Preoperative diagnosis, neoadjuvant and adjuvant chemoradiation, target and hormone therapies still raise many controversies. Disagreements about the role and type of surgical treatment are also often observed in medical literature. There are still insufficient data about the role of pelvic lymph node dissection and fertility-sparing surgery. Pathologists’ experience is of paramount importance for an accurate diagnosis. Additionally, genetics examinations become part of diagnosis in some sarcomas of the uterine corpus. Some gene mutations observed in uterine sarcomas are associated with different outcomes. Therefore, a development of molecular classification of uterine sarcomas should be considered in the future. In this review, we focus on the epidemiology, pathogenesis, pathology, diagnosis and treatment of the following sarcomas of the uterine corpus: leiomyosarcoma, low- and high-grade endometrial stromal sarcomas, undifferentiated sarcoma and adenosarcoma. Uterine carcinosarcomas are excluded as they represent an epithelial tumor rather than a true sarcoma.

Pulmonary Metastasizing Low-Grade Endometrial Stromal Sarcoma: Case Report and Review of Diagnostic Pitfalls

“…Since HG-ESS may contain an LG-ESS area, the whole area should be carefully examined; additionally, the observation of necrosis and increased mitosis favors an HG-ESS diagnosis. Finally, both LG-ESS and HG-ESS are known to have characteristic genetic changes (for LG-ESS, fusions of JAZF1-SUZ12 , JAZF1-PHF1 ; for HG-ESS, fusions of YWHAE-NUTM2, ZC3H7B-BCOR ); hence, it would be helpful to conduct a molecular test, if possible [ 26 , 27 , 28 ].…”

Maligne mesenchymale Uterustumoren

“…Makroskopisch präsentieren sich die LG-ESS als weiche, gelbe Tumoren, häufig mit hämorrhagischen und nekrotischen Arealen, die in der histologischen Untersuchung kleine und runde Zellen aufweisen. Sie sind häufig durch infiltratives Wachstum ins Myometrium charakterisiert und mit LVSI (lymphovascular Space Invasion) assoziiert [7,13]. In der Immunhistochemie sind die LG-ESS positiv für CD10, Caldesmon, Desmin und zeigen häufig eine starke Expression von Östrogen-und Progesteronrezeptoren [7,26].…”

“…Sie sind häufig durch infiltratives Wachstum ins Myometrium charakterisiert und mit LVSI (lymphovascular Space Invasion) assoziiert [7,13]. In der Immunhistochemie sind die LG-ESS positiv für CD10, Caldesmon, Desmin und zeigen häufig eine starke Expression von Östrogen-und Progesteronrezeptoren [7,26]. Die molekularpathologische Analyse zeigt in ca.…”

Novel Therapeutics in the Treatment of Uterine Sarcoma

“…However, in many clinical scenarios, there remains a lack of prospective evidence to inform the optimal therapeutic approach to these diseases. 2,3 Here, we describe recent advances in the treatment of advanced uterine sarcoma. A subset of ongoing clinical trials for patients with uterine sarcoma is highlighted in the Table.…”