Smooth Muscle Tumors of the Female Genital Tract

Smooth muscle tumors are the most common mesenchymal tumors of the female genital tract. However, awareness of tumor variants and unconventional growth patterns is critical for appropriate classification and patient management. For example, recognition of fumarate hydratase–deficient leiomyomas allows pathologists to alert providers to the potential for hereditary leiomyomatosis and renal cell carcinoma. Furthermore, myxoid and epithelioid smooth muscle tumors have different thresholds for malignancy than spindled tumors and should be classified by criteria specific to these variants. This article provides an overview of smooth muscle tumors of each major organ of the gynecologic tract and discusses diagnostic challenges.