Solitary extrapulmonary lymphangioleiomyomatosis of the liver: A case report and literature review.

Lymphangioleiomyomatosis (LAM) is a progressive disorder of unknown etiology that predominantly affects the lungs, and the resulting respiratory failure can be fatal. Extrapulmonary LAM is a rare disease that often occurs concurrently with pulmonary LAM. Usually, extrapulmonary LAM presents as a localized, well-circumscribed mass. Due to the rare occurrence of extrapulmonary LAM and its atypical location, extrapulmonary LAM is often difficult to diagnose prior to surgery. The present study describes the clinicopathological features of primary liver LAM in a 26-year-old woman. To the best of our knowledge, the present study presents the first case of a primary solitary extrapulmonary form of LAM in the liver.