Gastroesophageal-vulvar leiomyomatosis presenting over the course of 20 years
Siegler, R.W.; Rothstein, R.I.; Beecham, J.B.; Dunn, J.L.
Archives of Pathology and Laboratory Medicine 120(12): 1141-1144
1996
ISSN/ISBN: 0003-9985 PMID: 15456181 Document Number: 455610
Gastroesophageal-vulvar leiomyomatosis represents a rare syndrome of diffuse smooth muscle proliferation primarily in the gastroesophagus and vulva. To our knowledge, there are 19 cases reported in the literature, 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 delineate the process. Leiomyomatosis appears to be a systemic disorder, with indolent proliferations of smooth muscle in areas that may not be clinically significant.