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KMID : 0357920020360010051
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Korean Journal of Pathology
2002 Volume.36 No. 1 p.51 ~ p.54
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Pulmonary Lymphangioleiomyomatosis and Micronodular Pneumocyte Hyperplasia associated with Tuberous Sclerosis;A Case Report
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Kim Gou-Young
Lee Ju-Hie
Park Yong-Koo
Kim Youn-Hwa
Park Jae-Hoon
Yang Moon-Ho
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Abstract
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Lymphangioleiomyomatosis (LAM) is characterized by a hamartomatous proliferation of smooth muscle cells in the lung, mediastium, and abdomen. In the lung, an abnormal proliferation of smooth muscle is seen along the airways, blood vessels, and lymphatics, resuliting in honeycombing of the lung. It occurs in 0.1-1% of tuberous sclerosis (TSC) patients. Micronodular pneumocyte hyperplasia (MNPH)is a rare but distinctive pumonary manifestation of TSC, and appears to be a hamartomatous proliferation of the type Ⅱ pneumocytes. We report a case of pulmonary LAM and MNPH associated with TSC and bilateral renal angiomyolipoma in a 26-year-old woman. Immunohistochemicalty, the spindle cells of LAM were positive for HMB-45, but the type Ⅱ pneumocytes of MNPH were negative.
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KEYWORD
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Lung Neoplasms, Tuberous Sclerosis
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