GASTROESOPHAGEAL-VULVAR LEIOMYOMATOSIS PRESENTING OVER THE COURSE OF 20 YEARS

Gastroesophageal-vulvar leiomyomatosis represents a rare syndrome of d iffuse smooth muscle proliferation primarily in the gastroesophagus an d vulva. To our knowledge, there are 19 cases reported in the literatu re, most associated with Alport's syndrome. We recently encountered a case not associated with Alport's syndrome, which had progressed over the course of 20 years. We used immunohistochemistry to further deline ate the process. Leiomyomatosis appears to be a systemic disorder, wit h indolent proliferations of smooth muscle in areas that may not be cl inically significant.