Review of the pathophysiology, diagnosis, and therapy of vulvar leiomyoma, a rare gynecological tumor

Abstract

The objective of this article is to discuss the pathophysiology, diagnosis, differential diagnosis, and therapy of vulvar leiomyoma. We performed a review of all English-language reports of vulvar leiomyoma published in PubMed from 1978 to 2015 using the following search terms: "vulval leiomyoma," "vulvar leiomyoma," "vulval smooth muscle tumor," and "external genitalia smooth muscle tumor." Vulvar leiomyomas, which are rare benign monoclonal tumors, most commonly occur in the fourth and fifth decades of life. The genetics of vulvar leiomyoma remain undefined. Three principal histological patterns have been identified: spindled, epithelioid, and myxoid. Imaging tests such as ultrasound, endoscopic ultrasound, and magnetic resonance imaging are used in diagnosis. Surgical excision is the only curative treatment for vulvar leiomyomas. Establishment of a full differential diagnosis list and correct final diagnosis before surgery are essential for optimal clinical management. Although recurrence of vulvar leiomyoma is extremely rare, long-term follow-up of all cases is advisable.

Keywords: Vulvar leiomyoma; diagnosis; gynecological tumor; histology; imaging; management; treatment.

Figure 1.

Low-magnification view of spindle-shaped cells evenly distributed in a myxoid stroma. Prominent vessels are apparent (original magnification, ×40).

Figure 2.

Representative leiomyosarcoma. (a) Fusiform-shaped pleomorphic cells with hyperchromatic nuclei and rare bizarre-shaped atypical cells. (b) Necrotic tumor fields. (c) Atypical mitosis. (d) Tumor cells exhibiting diffuse positive staining for calponin.