Multiple vascular leiomyomas involving bilateral adrenal glands, spleen, and epicardium, associated with bilateral testicular microlithiasis and empty sella turcica

Abstract

Leiomyomas can arise from any tissue having smooth muscle as one of its constituents, especially gastrointestinal and genitourinary systems, less frequently in the skin, and rarely in deep soft tissue. An unusual case of multiple vascular leiomyomas involving bilateral adrenal glands, spleen, and epicardium, associated with bilateral testicular microlithiasis, and empty sella turcica is reported in a 15-year-old boy. The adrenal masses were incidentally discovered by ultrasonography. These three findings, taken individually are rare, and the constellation of the th ree, to the best of our knowledge, is the first recorded example. Furthermore, bilateral involvement of the adrenal glands with leiomyomas is, by itself, very uncommon. The tumors considered benign were surgically removed. Orchiectomy wasn't performed. The boy is well after 1.5-year follow-up under cortisone, 9 alpha-fludrocortisone, and testosterone therapy. Copyright (C) 1997 by W.B. Saunders Company.