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.

Highlights

  • Sarcomas of the uterine corpus are malignant neoplasms, which are further classified into mesenchymal tumors and mixed tumors [1,2]

  • 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

  • Molecular studies are more often used as an indispensable part of uterine sarcoma diagnosis and prognosis

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Summary

Introduction

Sarcomas of the uterine corpus are malignant neoplasms, which are further classified into mesenchymal tumors and mixed (epithelial plus mesenchymal) tumors [1,2]. The low frequency of these malignancies implies insufficient experience in the diagnosis, incomplete surgical and complex treatment. 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. Its annual incidence rate is approximately 0.8 per 100,000 women It is the most common type of uterine sarcomas originating from the myometrium [3,4,5,6]. It is more common among people of the African-American race, those that have been exposed to prolonged use of tamoxifen for over 5 years and women over 40 years of age, with incidence increasing rapidly after age 50 [5,7]. There is an increased risk of ULMS for patients with p53 gene mutations, for those who have been exposed to radiotherapy for childhood cancers, and those with certain inherited genetic syndromes, including hereditary retinoblastoma and Li-Fraumeni syndrome [5,6,7]

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