Matsuyama Atsuji, Hisaoka Masanori, Ichikawa Kazuhito, Fujimori Takahiro, Udo Kazuma, Uchihashi Kazuyoshi, Aoki Shigehisa, Hashimoto Hiroshi
Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
Pathol Int. 2008 May;58(5):306-10. doi: 10.1111/j.1440-1827.2008.02228.x.
Presented herein are two unusual epithelioid angiomyolipomas (AML) displaying prominent stromal sclerosis. Both patients were middle-aged women without a clinical history of tuberous sclerosis. One patient (case 1) had a 2 cm lesion arising in the renal cortex, and another (case 2) had a pararenal retroperitoneal tumor measuring 13 cm. Both tumors were composed of sheets or nests of polygonal epithelioid or short spindle cells having uniform round to oval nuclei and eosinophilic cytoplasm with cords of hyalinized sclerotic stroma between them. The tumor in case 2 had small areas of mature-looking fat cells. Immunohistochemically, epithelioid tumor cells were diffusely positive for actins and desmin in both cases, and melanoma antigen recognized by T cells (MART)-1 was positive in patient 2. Scattered HMB-45-immunoreactive cells were identified in the sclerotic cords of both tumors, but epithelioid tumor cells were essentially negative for HMB-45. The characteristic clinicopathological and immunohistochemical features of the present cases are analogous to a subset of epithelioid AML or sclerosing perivascular epithelioid cell tumors previously reported.
本文报道了两例罕见的上皮样血管平滑肌脂肪瘤（AML），其显示出显著的间质硬化。两名患者均为中年女性，无结节性硬化症的临床病史。一名患者（病例1）肾皮质出现一个2 cm的病灶，另一名患者（病例2）有一个13 cm的肾旁腹膜后肿瘤。两个肿瘤均由多边形上皮样或短梭形细胞的片状或巢状结构组成，细胞核呈均匀的圆形至椭圆形，细胞质嗜酸性，其间有透明化硬化间质索。病例2的肿瘤有小面积外观成熟的脂肪细胞。免疫组织化学检查显示，两例病例中的上皮样肿瘤细胞肌动蛋白和结蛋白均弥漫性阳性，病例2中T细胞识别的黑色素瘤抗原（MART）-1呈阳性。在两个肿瘤的硬化索中均发现散在的HMB-45免疫反应性细胞，但上皮样肿瘤细胞HMB-45基本为阴性。本病例的特征性临床病理和免疫组织化学特征与先前报道的上皮样AML或硬化性血管周上皮样细胞瘤的一个亚组相似。
