Current status on treatment of uterine adenosarcoma: updated literature review

Adenosarcoma is a rare subtype of uterine sarcoma, composed by a combination of a benign epithelial and a malignant, but generally low grade, mesenchymal component and it is considered the least aggressive form of its epithelial counterpart, uterine carcinosarcoma. The presence of myometrial invasion and the extent of disease outside the uterus determines the stage of disease, while the presence of sarcomatous overgrowth represents the most important histological prognostic factor, correlating with the grade of disease. Management of localized uterine adenosarcoma (UAS) is based on complete surgical removal. Cytotoxic chemotherapy with doxorubicin-based regimens, gemcitabine/docetaxel, trabectedin or platinum-based regimens remains the standard of care for the advanced stage, recurrent or metastatic adenosarcoma, although an integrated approach of surgery and medical therapy should also be considered in this setting. Hormone therapy represents a potential therapeutic option for estrogen receptor (ER) and progesterone receptor (PR) positive low grade adenosarcoma. Because of the extreme rarity of UAS, most available literature data arise from retrospective case series and reports, while uniform clinical guidelines are still lacking. The aim of this updated literature review is to better define the multidisciplinary management of this rare neoplasm, focusing on the role of surgical and medical treatment and proposing a treatment flow sheets that could help to guide our clinical practice.