Bilateral ovarian lymphangioma (Lymphangioleiomyoma)

Lymphangiomas of the ovary are rare and are usually unilateral. We present a 50-yr-old patient who presented with irregular bleeding secondary to multiple uterine leiomyomas who was found to have bilateral ovarian lymphangiomas. There was no evidence of pelvic lymphatic obstruction or of lymphadenopathy, and this appeared to exclude the possibility of acquired lymphangiectasia. The ovarian tumors were associated with a prominent smooth muscle cell component that partly surrounded many of the dilated vascular spaces to the extent that the diagnoses of lymphangioleiomyoma and lymphangioleiomyomatosis were also considered. However, there was no clinical evidence of lymphangioleiomyomatosis in other sites and the smooth muscle cells did not express melanocytic markers immunohistochemically. Lymphangioma and lymphangioleiomyoma should be considered in the differential diagnosis of bilateral, multicystic ovarian neoplasms. © 2013 International Society of Gynecological Pathologists.