Introduction

Uterine sarcomas are a rare and aggressive group of malignancies that arise from the mesenchymal tissue of the uterus.  They account for approximately 3%-7% of all uterine cancers and have a poor prognosis compared to endometrial carcinomas.

Pathogenesis

The most common uterine malignancy (endometrial cancer) arises from the endometrium and tends to be more amenable to treatment.  In contrast, uterine sarcoma—a broad term that encompasses multiple malignancies—arises from the myometrial or stromal elements of the uterus.  They are rare, aggressive, and difficult to treat.

Uterine sarcomas arise from genetic mutations in tumor suppressor genes (eg, TP53, PTEN) and oncogenes, leading to unregulated cellular proliferation within the uterine mesenchyme.  Uterine sarcomas arise independently, not from benign leiomyomas (fibroids).

Risk factors

  • Long-term tamoxifen use (>5 years):  Associated with an increased risk; however, the absolute risk is low.

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Tables

Table 1