Lymphangiomyoma: Clinicopathologic study and ultrastructural confirmation of its histogenesis

Abstract

Two new cases of lymphangiomyoma are added to 28 cases previously reported. One of these was studied ultrastructurally, with findings that support the designation “lymphangiomyoma,” i.e., smooth muscle and lymphatic channels. The disease seems to occur exclusively in females, is not infrequently associated with endocrine disorders, has some features analogous to tuberous sclerosis, and may represent an incomplete expression or forme fruste of Bourneville's disease. The condition consists of one or more nodules of tumor‐like proliferation of smooth muscle fascicles, divided from each other by lymphatic channels, and involves major lymphatic trunks in the retroperitoneum or mediastinum, lymph nodes in these regions and, in about two thirds of cases, lymphatics of the lungs. The most common complaints are referable to chylous effusions, pulmonary insufficiency, and pneumothorax. Three patients have been followed for over 14 years after excision of the nodules and postoperative radiotherapy. Copyright © 1973 American Cancer Society