Abstract
Simple SummaryUterine sarcomas are rare mesenchymal malignant cancers, and surgery represents the mainstay of treatment for early-stage disease. In metastatic setting, uterine sarcomas’ treatment includes palliative surgery, a metastases resection, chemotherapy and targeted therapy. Hormonal therapies may also represent an effective option. Frequently, ER and PR are highly expressed in uterine sarcomas patients and they represent a favourable prognostic factor associated with improved overall survival. The scope of the present review is to report the existing evidence and future perspectives on hormonal therapy in uterine sarcomas, with a special focus on aromatase inhibitors, progestins and gonadotropin-releasing hormone analogues, in order to clarify their potential role in daily clinical practice.Uterine sarcoma (US) is a rare mesenchymal malignant cancer type, accounting for 3–7% of uterine malignancies. US prognosis is still poor due to high local and distant recurrence rates. As for molecular features, US may present variable oestrogen receptor (ER) and progesterone receptor (PR) expressions, mostly depending on histotype and grading. Surgery represents the mainstay of treatment for early-stage disease, while the role of adjuvant chemotherapy or local radiotherapy is still debated and defined on the basis of histotype, tumour grading and stage. In metastatic setting, uterine sarcomas’ treatment includes palliative surgery, a metastases resection, chemotherapy, hormonal therapy and targeted therapy. As for the chemotherapy regimen used, drugs that are considered most effective are doxorubicin (combined with ifosfamide or alone), gemcitabine combined with docetaxel and, more recently, trabectedin or pazopanib. Hormonal therapies, including aromatase inhibitors (AIs), progestins and gonadotropin-releasing hormone analogues (GnRH-a) may also represent an effective option, in particular for low-grade endometrial stromal sarcoma (LGESS), due to their favourable toxicity profile and patients’ compliance, while their role is still under investigation in uterine leiomyosarcoma (uLMS), high-grade endometrial stromal sarcoma (HGESS), undifferentiated uterine sarcoma (USS) and other rarer US. The present review aims to analyse the existing evidence and future perspectives on hormonal therapies in US, in order to clarify their potential role in daily clinical practice.
Highlights
Uterine sarcomas (US) are rare mesenchymal malignant tumours, accounting for about 3–7% of all uterine malignancies and less than 1% of all malignancies from the female genital tract [1].According to the updated World Health Organization (WHO, fifth edition) classification, they can be distinguished based on their histology as uterine leiomyosarcoma, low-grade endometrial stromal sarcoma (LGESS), high-grade endometrial stromal sarcoma (HGESS), undifferentiated uterine sarcoma (UUS), adenosarcoma, rhabdomyosarcoma and perivascular epithelioid cell tumour (PEComa)
Type of published study: clinical trial, review articles, guidelines; Selected population: patients affected by uterine sarcomas both in early-stage or advanced disease; Evaluation of ER and/or progesterone receptor (PR) expression; No limitation regarding the year of the publication of papers was used
LGESS have a better prognosis than HGESS and are more common [13]
Summary
Uterine sarcomas (US) are rare mesenchymal malignant tumours, accounting for about 3–7% of all uterine malignancies and less than 1% of all malignancies from the female genital tract [1]. According to the updated World Health Organization (WHO, fifth edition) classification, they can be distinguished based on their histology as uterine leiomyosarcoma (uLMS), low-grade endometrial stromal sarcoma (LGESS), high-grade endometrial stromal sarcoma (HGESS), undifferentiated uterine sarcoma (UUS), adenosarcoma, rhabdomyosarcoma and perivascular epithelioid cell tumour (PEComa). ULMS is the most common, whereas adenosarcoma, rhabdomyosarcoma, and perivascular epithelioid cell tumour (PEComa) are extremely rare. Carcinosarcomas (malignant mixed müllerian tumours, MMMTs) are no longer considered as sarcomas, as they are dedifferentiated carcinomas with both epithelial and stromal components; nowadays, they are considered, staged and treated as high-grade endometrial cancers [2]. Distant metastases may occur even at an early stage, and distant relapse after radical surgery is common
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