Malignant Monotypic Epithelioid Angiomyolipoma of the Retroperitoneum

International Journal of Surgical Pathology - Tập 11 Số 3 - Trang 223-228 - 2003
Sean K. Lau1, Alberto M. Marchevsky2, Robert J. McKenna3, Daniel Luthringer2
1Division of Pathology, City of Hope National Medical Center, Duarte, California
2Department of Pathology and Laboratory Medicine and Cedars-Sinai Medical Center, Los Angeles, California
3Thoracic Surgery, Cedars-Sinai Medical Center, Los Angeles, California

Tóm tắt

Monotypic epithelioid angiomyolipoma (EAML) is an uncommon variant of angiomyolipoma, occurring primarily in the kidney and characterized by a predominance of HMB-45-positive epithelioid cells. EAML is generally considered a benign neoplasm, though rare cases exhibiting malignant behavior have been reported. We describe a patient with a primary retroperitoneal monotypic EAML, composed exclusively of atypical epithelioid cells, which metastasized to the liver and the mediastinum. This case represents, to our knowledge, the first description of metastatic disease occurring in an EAML of this particular site

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