Govender Dhirendra, Sabaratnam Rathi M, Essa Ahmed S
Department of Pathology, Nelson R. Mandela School of Medicine, University of Natal, Durban, South Africa.
Am J Surg Pathol. 2002 May;26(5):670-5. doi: 10.1097/00000478-200205000-00014.
A group of lesions show morphologic and immunophenotypic evidence of differentiation toward a putative perivascular epithelioid cell. These so-called PEComas include angiomyolipoma, lymphangiomyoma, lymphangioleiomyomatosis, renal capsuloma, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and clear cell "sugar" tumor. PEComas are characterized by strong immunoreactivity with the HMB45 antibody and variable expression of muscle markers. This family of lesions may be composed of a spectrum of cells from epithelioid to spindle cells with clear to granular eosinophilic cytoplasm. One member of this family, composed of epithelioid cells with glycogen-rich clear cytoplasm, is descriptively called a clear cell "sugar" tumor. This tumor, originally described in the lung, is being recognized increasingly in extrapulmonary sites. We report a case of a primary extrapulmonary clear cell "sugar" tumor occurring in the right breast of a 16-year-old girl. The tumor was composed of clear epithelioid cells with abundant glycogen and distinct cell borders. The tumor showed strong immunoreactivity with HMB45 antibody and Melan-A. There was focal vimentin staining. In addition, there was diffuse and strong nuclear staining for progesterone receptor. Antibodies to actins, S-100 protein, cytokeratins (AE1/AE3 and CAM5.2), desmin, and estrogen receptor were negative. The tumor was completely excised, and the patient is well without evidence of disease 9 months postexcision.
一组病变显示出向假定的血管周围上皮样细胞分化的形态学和免疫表型证据。这些所谓的PEComas包括血管平滑肌脂肪瘤、淋巴管肌瘤、淋巴管平滑肌瘤病、肾包膜瘤、镰状韧带/圆韧带透明细胞肌黑色素细胞瘤和透明细胞“糖”瘤。PEComas的特征是对HMB45抗体有强免疫反应性，且肌肉标志物表达各异。这一系列病变可能由从上皮样细胞到梭形细胞的多种细胞组成，细胞质从透明到嗜酸性颗粒状。该系列病变中的一个成员，由富含糖原的透明细胞质的上皮样细胞组成，被描述性地称为透明细胞“糖”瘤。这种肿瘤最初在肺部被描述，现在在肺外部位越来越多地被识别出来。我们报告了一例发生在一名16岁女孩右乳腺的原发性肺外透明细胞“糖”瘤病例。肿瘤由具有丰富糖原和明显细胞边界的透明上皮样细胞组成。肿瘤对HMB45抗体和Melan-A有强免疫反应性。有局灶性波形蛋白染色。此外，孕激素受体有弥漫性强核染色。肌动蛋白、S-100蛋白、细胞角蛋白（AE1/AE3和CAM5.2）、结蛋白和雌激素受体的抗体均为阴性。肿瘤被完全切除，患者在切除后9个月情况良好，无疾病迹象。
