Multicentric renal angiomyolipoma associated with pulmonary lymphangioleiomyomatosis: case report, with histologic, immunohistochemical, and DNA content analyses.

A 26-year-old pregnant woman presented with pulmonary lymphangioleiomyomatosis (LAM) and multicentric angiomyolipoma (AML) involving the left kidney and perirenal lymph nodes. In both tumors, smooth muscle cells were the predominant component. Immunohistochemically, these cells stained for vimentin, smooth muscle actin, desmin, and HMB-45. Estrogen receptors were weakly positive in LAM and negative in AML. Progesterone receptors were negative for both lesions. DNA content analysis studies showed the renal AML to be diploid with a minor aneuploid component, the lymph node AML aneuploid, and the LAM diploid. The diagnostic and prognostic significance of hormonal studies and DNA content analysis are discussed.