Journal of Pathology and Translational Medicine

Case Reports

Pulmonary Lymphangioleiomyomatosis and Micronodular Pneumocyte Hyperplasia associated with Tuberous Sclerosis: A Case Report.: Gou Young Kim, Juhie Lee, Yong Koo Park, Youn Wha Kim, Jae Hoon Park, Moon Ho Yang; Korean J Pathol. 2002;36(1):51-54.

Abstract PDF: 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, resulting 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 pulmonary 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. Immunohistochemically, the spindle cells of LAM were positive for HMB-45, but the type II pneumocytes of MNPH were negative.

Pulmonary Lymphangioleiomyomatosis: A case report.: Won Bo Jo, Nam Hee Won, Seung Yong Paik, Hae Kyung Ahn; Korean J Pathol. 1991;25(3):269-274.

Abstract PDF: 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.

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