Pulmonary Lymphangioleiomyomatosis: A case report.
- Author:
Won Bo JO
;
Nam Hee WON
;
Seung Yong PAIK
;
Hae Kyung AHN
- Publication Type:Case Report
- Keywords:
Lymphangioleiomyomatosis;
Lung;
Tuberous sclerosis
- MeSH:
Female;
Humans;
Adenoma
- From:Korean Journal of Pathology
1991;25(3):269-274
- CountryRepublic of Korea
- Language:Korean
-
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.
