Lau Sean K, Marchevsky Alberto M, McKenna Robert J, Luthringer Daniel J
Division of Pathology, City of Hope National Medical Center, Duarte, California, USA.
Int J Surg Pathol. 2003 Jul;11(3):223-8. doi: 10.1177/106689690301100313.
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.
单型上皮样血管平滑肌脂肪瘤（EAML）是血管平滑肌脂肪瘤的一种罕见变体，主要发生于肾脏，其特征为HMB-45阳性上皮样细胞占优势。EAML通常被认为是一种良性肿瘤，不过已有罕见病例报道其具有恶性行为。我们描述了一名原发性腹膜后单型EAML患者，该肿瘤仅由非典型上皮样细胞组成，已转移至肝脏和纵隔。据我们所知，该病例是对发生于这一特定部位的EAML转移性疾病的首次描述。
