Lymphangiomyomatosis: A clinico-anatomical entity

Abstract

A 65-year-old woman presenting with progressive dyspnea and chylous ascites is reported. Necropsy revealed the presence of masses of spindle cells in and around the walls of lymph vessels, and massively involving the retro-peritoneal and to a lesser extent the mediastinal lymph nodes. Honeycombing of the lungs was associated with similar spindle-cell masses arising from pulmonary lymph vessels. These findings are those of a disease known as lymphangiomyomatosis, which has heretofore been characterized by infiltration and obstruction of the thoracic duct, and by chylothorax. In this case, there was no chylothorax but only chylous ascites; furthermore the thoracic duct was patent. These findings argue against the reactive origin of the spindle-cell proliferation and suggest that these formations are of hamartomatous nature.