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Pulmonary lymphangioleiomyomatosis followed by a localized retroperitoneal lymphangioleiomyoma: Case report

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Tai HC, Lin JW, Wang YH, Eng HL, Lu HI, Liu YW, Huang CC. Pulmonary lymphangioleiomyomatosis followed by a localized retroperitoneal lymphangioleiomyoma. APMIS 2006;114:821–4.

Lymphangioleiomyomatosis is a rare disease that affects females of reproductive age. Microscopically, it is characterized by abnormal proliferation of immature smooth muscle-like cells that grow diffusely in the lung. Extrapulmonary manifestations in the mediastinum, peritoneum and pelvic lymph nodes are uncommon. We here describe a patient who initially presented with pulmonary lymphangioleiomyomatosis and subsequently developed a localized retroperitoneal mass. Pathologic examination showed that the mass was a lymphangioleiomyoma. The result of the immunohistochemical study was the same as that for the pulmonary lesion. It is therefore suggested that metastatic lymphangioleiomyoma should be included in the differential diagnosis in the patient with pulmonary lymphangioleiomyomatosis presenting with an extrapulmonary lesion.

Keywords: Lymphangioleiomyomatosis; lung; metastasis; retroperitoneum

Document Type: Research Article


Affiliations: 1: Department of Pathology, Changhua Christian Hospital, Changhua, and Departments of 2: Pathology, 3: Internal Medicine, and 4: Surgery, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan

Publication date: November 1, 2006


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