Smooth Muscle Tumors of the Female Genital Tract

Smooth muscle tumors are the most common among mesenchymal tumors in the female genital tract. The vast majority of these neoplasms are clinically benign and easy to diagnose. In contrast, leiomyosarcomas are highly aggressive tumors that may pose considerable diagnostic problems when they display unusual (myxoid or epithelioid) morphology, ambiguous histologic features for malignancy, or an unusual anatomic distribution. Diagnostic criteria for these problematic tumors vary depending on the site and type of histologic differentiation, and are based on a combination of 3 major criteria: (1) moderate to severe cytologic atypia; (2) increased mitotic index; and (3) tumor cell necrosis. Certain benign smooth muscle proliferations may show worrisome histologic features or unusual growth patterns, causing concern for leiomyosarcoma. Furthermore, other tumors, including perivascular epithelioid tumors, may mimic leiomyosarcoma. Careful attention to the clinical and anatomic setting, cytologic and architectural features, and immunohistochemical characteristics are helpful in distinguishing these entities. This article discusses conventional smooth muscle tumors as well as unusual subtypes, with emphasis on the diagnostic criteria and problems in differential diagnosis that arise at each site within the female genital tract.