A case of systemic lymphangioleiomyomatosis

Abstract

A rare case of systemic lymphangioleiomyomatosis in a 31-year female is discussed. Clinically and radiologically, there was a 3-year progression of the disease (relapsing bilateral pneumothorax with a chyle fluid, dynamic change of CT-data). The diagnosis was verified after histological and immunohistochemical study. The patient was effectively treated with Everolimus. Clinical, radiological and histological data suggest lymphangioleiomyomatosis is a hormone-related disease.