[Pulmonary lymphangiomyomatosis associated with spontaneous pneumothorax]

A 34-year-old female associated with spontaneous pneumothorax was diagnosed as pulmonary lymphangiomyomatosis by open lung biopsy and pathological examination. The biopsy specimen of the lung showed marked proliferation of smooth muscle in the lung. This disease is seen only in women in childbearing age, and hormonal therapy is ineffective, but bilateral oophorectomy seems, in some cases, to have a beneficial effect. Lung transplantation can be applied to patients with terminal pulmonary lymphangiomyomatosis.