Lymphangiomyoma and tuberous sclerosis

A patient with tuberous sclerosis and biopsy-proven lymphangiomyoma is presented. Lymphangiomyoma is a hamartomatous proliferation of predominantly smooth muscle that involves the lung and lymph nodes. It gives a syndrome of chylothorax, diffuse pulmonary reticular pattern, and frequent spontaneous pneumothorax. That the pulmonary involvement of tuberous sclerosis is practically identical, both grossly and microscopically, to that of lymphangiomyoma and that both syndromes occur in females between 20 to 50 years of age, suggests a common pathologic basis. The concept that lymphangiomyoma is a hamartoma makes this association an attractive one. The presence of chylothorax has been emphasized as a possible differential point between these two entities. However, 2 cases of tuberous sclerosis with chylothorax have now been reported. The presence or absence of chylothorax may depend on whether the thoracic duct is involved.