Lymphangiomyoma, a benign lesion of chyliferous lymphatics synonymous with lymphangiopericytoma

Abstract

Six cases are presented of an entity which previously had been described as lymphangiopericytoma, but which now are considered to be more correctly termed “lymphangiomyoma” An additional 14 cases from the literature are reviewed. The lesion, which shows a striking female sex predominance, often involves the thoracic duct, has an almost constant association with chylothorax and a frequent one with pulmonary disease consisting of lymphangiectasis, honeycombing with proliferation of smooth muscle and atypical lipid pneumonia, possibly of chylous origin. No lesion has metastasized to distant organs or invaded organs locally and the authors conclude that the lesion is best considered as a hamartoma, often of multifocal origin. A close relationship is noted between lymphangiomyoma and certain secondary lesions associated with tuberous sclerosis. The clinical course and response to therapy are described.