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HOME > J Pathol Transl Med > Volume 25(3); 1991 > Article
Case Report Pulmonary Lymphangioleiomyomatosis: A case report.
Won Bo Jo, Nam Hee Won, Seung Yong Paik, Hae Kyung Ahn
Journal of Pathology and Translational Medicine 1991;25(3):269-274
DOI: https://doi.org/
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1Department of Pathology, Korea University Medical College, Seoul, Korea.
2Department of Laboratory Medicine, Korea University Medical College, Seoul, Korea.
3KangNam SungShim Hospital, Seoul, Korea.

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.


JPTM : Journal of Pathology and Translational Medicine