Gastroesophageal leiomyomatosis in a young women with vulvar leiomyomatosis and gallstone disease: report of a rare case

ABSTRACT

Gastroesophageal-vulvar leiomyomatosis is a very rare condition; it is characterized by diffuse, ill-defined proliferation of smooth muscle in the esophagus and vulva. We present an interesting case of esophageal leiomyomatosis in a woman with a history of vulvar leiomyomatosis and a gall bladder full of stones. She was I8-years old and had a 4 year history of vulvar mass and clitoromegaly, with subclinical dysphagia. This paper represents the first reported simultaneous occurrence of these three pathologic entities in the English literature. Esophago-vulvar leiomyomatosis should be considered in a young patient with vulvar mass and long-standing dysphagia in whom a smooth, tapered esophageal narrowing on barium study and circumferential esophageal wall thickening on CT scan are seen. An esophagectomy combined with a reconstruction procedure is indicated