Lymphangiomyomatosis--newer concepts in pathogenesis and management--case reports.

Lymphangiomyomatosis was first reported more than forty years ago. Although its incidence is rare, its occurrence is being increasingly recognized and has been the subject of a growing number of cases reports in recent years. This study adds 2 more cases to the file. Both cases involved young women with the characteristic symptoms of dyspnea, cough, abdominal discomfort and swelling, chest pain, and hemoptysis, with abundant formation of refractory chylous, serous ascites. Although the progression of the disease differed in each case, pulmonary function was affected in a similar way by the presence of obstructive and restrictive defects and a decrease in diffusing capacity. Underlying abnormalities were dilated lymphatics, thickened lymphatic walls, and muscular proliferation of leiomyomatous origin, leading to bronchial restriction. The authors point to the subtlety required in arriving at a differentiated diagnosis of lymphangiomyomatosis lymphangioleiomyomatosis and the difference between the two conditions. They also make particular recommendations in respect to the importance of preliminary hormone receptor tests and to the wisdom to be exercised in ligating a main lymphatic duct in the chest to control the formation of ascites. Pneumothorax, a frequent manifestation of lymphangiomyomatosis, is found to be the result of chronic air trapping due to a combination of narrowing of conducting airways and disruption of normal lung parenchyma.

Pulmonary lymphangiomyomatosis.

Lymphangiomyomatosis is an interesting disease with distinctive clinical and histopathologic findings. We report herein two additional cases of lymphangiomyomatosis, including one with clinical improvement after therapy with progesterone. This case is of particular significance in view of the patient's negative sex steroid receptor analysis. These findings open new avenues for future considerations in the therapy of this unusual but interesting disease.