An Autopsy Case of Pulmonary Lymphangiomyomatosis Unresponsive to Various Anti-Estrogen Therapies

Abstract

A 26-year-old woman was admitted to our hospital complaining of progressive dyspnea and hemoptysis with a past history of chylothorax and recurrent pneumothorax. The diagnosis of pulmonary lyphangiomyomatosis was made by transbronchial lung biopsy.
She was first treated with intramuscular progesterone therapy unsuccessfuly. Thereafter she received lineac irradiation (14Gy) in the bilateral ovarian regions and oral tamoxifen therapy in addition to progesterone. Despite these treatments her clinical condition deteriorated and refractory chyrous ascites developed. She underwent peritoneo-jugular shunt and bilateral oophorectomy. Seven days after operation she suddenly died of respiratory failure due to pneumothorax. At autopsy, microscopic examination revealed abundant proliferation of smooth muscle in the pleura, walls of blood vessels and bronchi, alveolar septa and acinar interstitial tissues. Neither estrogen-receptor nor progesterone-receptor was detected in the pulmonary tissue obtained at autopsy.