Uterine leiomyosarcoma: A review of the literature and update on management options

Uterine leiomyosarcoma is the most common type of uterine sarcoma. It is an extremely aggressive malignancy associated with a poor overall prognosis. Women affected may vary in age, but are most often diagnosed in their perimenopausal years. Presenting symptoms may be vague and mimic other benign uterine conditions. Preoperative diagnosis of leiomyosarcoma is difficult and often only made at time of surgical resection. These rare mesenchymal tumors are characterized by cytologic atypia, a high mitotic index, and tumor necrosis on histologic inspection. Management of early stage disease entails hysterectomy and complete surgical resection of gross tumor, though routine oophorectomy or lymph node dissection do not appear to confer much clinical benefit. Adjuvant therapy for early stage disease remains controversial as multiple clinical trials have failed to demonstrate benefit on overall survival. Recently, progress has been made in regards to therapy for advanced and recurrent disease. Novel chemotherapeutics, targeted therapies such as olaratumab and pazopanib, and new immunotherapies such as nivolumab and pembrolizumab have demonstrated promise in these previously difficult drug-resistant patients. In this article, we provide a detailed review of uterine leiomyosarcoma including epidemiology, clinical presentation, diagnosis, and pathologic characteristics. We then go on detail management strategies, including options for adjuvant therapy, and highlight new and developing regimens in the field.