Journal of Pathology and Translational Medicine

Case Report

Intrathoracic Lymphangiomatosis: An autopsy case report.: Eunmee Han, Kyu Rae Kim, In Chul Lee; Korean J Pathol. 2000;34(2):154-159.

Abstract PDF: We report a rare case of intrathoracic lymphangiomatosis associated with chylothorax in a 3-year-old boy. The patient had been healthy until he had chickenpox followed by continued dyspnea. The biopsy specimen showed proliferating lymphatic channels & spindle cells in the lung tissue. The boy died of respiratory failure despite conservative treatment and surgical treatment with pleurodesis. An autopsy was performed. Pleura and pericardium showed severe adhesion to the right lung parenchyma. The cut surface of lung showed thickened interlobular septum with honeycomb-appearance. Histologically, extensive intercommunicating and anastomosing endothelial-lined lymphatic channels were noted along the visceral and parietal pleura, pericardium, around the great vessels, and in the anterior mediastinum over the thymus. These lymphatic channels infiltrated into the pulmonary parenchyme along the bronchovascular bundles. There were scattered areas of spindle cell proliferation with extravasation of RBCs mimicking Kaposi's sarcoma. Histologic diagnosis and differential diagnosis on biopsy materials can be difficult to establish but awareness of the intrathoracic lymphangiomatosis and its various clinical presentation may be helpful for diagnosis.

Original Article

Pulmonary Lymphangioleiomyomatosis: Pathologic Analysis of Eight Korean Cases.: Seung Sook Lee, Jeong Wook Seo, Eul Keun Ham, Yong Il Kim, Nam Hee Won, Jung Gi Im, Young Soo Shim; Korean J Pathol. 1994;28(4):358-367.

Abstract PDF: Histopathology of pulmonary lymphangioleiomyomatosis(LAM) is studied using four new cases and six previously reported cases, which include two cases without definite evidence of LAM. The important diagnostic features of this lesion were nodular proliferation of immature smooth muscle and cleft or cyst formation within the nodules of smooth muscle cells. The nuclei of the smooth muscle cells were bigger than those of blood vessels or fibrotic lung, and the direction of nuclei was irregular. The lung parenchyma showed little inflammatory change but there were multiple air cysts with smooth muscle nodules at their margin. There were two cases with exuberant proliferation of smooth muscle nodules and two cases with papilliferous projections of the cells into lymphatic lumen. Whereas, three cases had only a few small slender nodules of smooth muscle cells at the margin of air cyst. The lymphatic lumen with smooth muscle nodules is dilated in four cases but other four cases show collapsed lumen. Pulmonary hemorrhage and hemosiderosis were prominent in three cases. There were variety of histology in terms of the cellularity of smooth muscle nodules, the size of the lymphatic lumen and the degree of pulmonary destruction, which may have significance on the clinical presentation and prognostication.

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