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The Korean Journal of Pathology 1991;25(3): 269-274.
Pulmonary Lymphangioleiomyomatosis: A case report.
Won Bo Jo, Nam Hee Won, Seung Yong Paik, Hae Kyung Ahn
1Department of Pathology, Korea University Medical College, Seoul, Korea.
2Department of Laboratory Medicine, Korea University Medical College, Seoul, Korea.
3KangNam SungShim Hospital, Seoul, Korea.
ABSTRACT
Lymphangioleiomyomatosis(LAM) is a rare disease of women of child-bearing age in which there is progressive hyperplasia of atypical smooth mucle along lymphatics in the lung, and/or axial lymphatics in the thorax and abdomen, resulting in honeycombing of lung. Interestingly there has been a speculation that it represents a forme furste or incomplete expression of tuberous sclerosis complex. This is based on the observation that patients with tuberous sclerosis can manifest pulmonary lesions indistinguishable from LAM. We report a case of LAM occuring in a 39-year-old female, who complained of recurrent pneumothorax, chest pain and shortness of breath. Three years ago, the patient had right nephrectomy under the diagnosis of ruptured angiomyolipoma. A X-ray film of the chest showed honeycombing with a diffusely reticulonodular pattern and cyst-like spaces. She had a characteristic facial appearance of adenoma sebaceum, which her father and uncle had. Microscopically, the lung showed a marked smooth muscle proliferation around the slit-like lymphatic spaces and also some respiratory bronchioles.
Key Words: Lymphangioleiomyomatosis; Lung; Tuberous sclerosis