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.