Uterine Sarcomas: How to Navigate an Ever-Growing List of Subtypes

Uterine sarcomas are rare mesenchymal tumors that are aggressive cancers. The rarity of these tumors, and consequently limited prospective data, has made surgical management of uterine sarcomas challenging. One major obstacle in the management of uterine sarcomas is establishing the diagnosis prior to surgery, which is crucial for appropriate intraoperative management. This paper serves to review aspects of surgical management of uterine sarcomas that remain unanswered. Distinguishing common benign myomas from rare uterine sarcomas is important for operative planning and subspecialty care because benign myomas are frequently managed with minimally invasive hysterectomy or myomectomy, whereas the mainstay of management of uterine sarcomas is hysterectomy without specimen fragmentation. Preoperative clinical presentation, serum studies, imaging, and histologic examination all have limitations in establishing a preoperative diagnosis. In addition, patients are often of reproductive age and desire fertility preservation. Although surgery remains the cornerstone for management, high-quality data guiding best practices are sparse. Morcellation should be avoided. Expert pathologic review, imaging to assess for metastatic disease, and consideration of hormone receptor testing are advisable. Recent data have further informed surgical approach and fertility preservation in early-stage disease, but controversy remains. Despite substantial advancement in the medical management of uterine sarcomas, surgical management of uterine sarcomas remain challenging. Larger studies with long-term follow-up are needed to guide fertility preservation surgery options, both local resection and ovarian preservation, further in young women. Development of novel methods to differentiate between benign and malignant uterine masses is needed.