Shi Huijuan, Cao Qinghua, Li Hui, Zhen Tiantian, Lai Yingrong, Han Anjia
Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University Guangzhou, China.
Int J Clin Exp Pathol. 2014 Aug 15;7(9):6357-63. eCollection 2014.
To report one case of malignant perivascular epithelioid cell tumor (PEComa) of the kidney with rare pulmonary and ileum metastases and analyze its clinicopathological features.
We analyzed the clinicopathological features of one case of malignant PEComa of the kidney with pulmonary and ileum metastases. Immunohistochemistry staining was performed.
The patient was a 48-year-old man with a renal mass approximately 14 cm × 11 cm × 8 cm in size. Microscopically, the tumor was mainly composed of polygonal epithelioid cells with dense eosinophilic cytoplasm and round nuclei with small nucleoli. Focal tumor cells showed pleomorphism with multinucleated giant cells and prominent nucleoli. The tumor cells nests were surrounded by thick-walled irregular blood vessels. Focal fat cells were found within the tumor. Hemorrhage and coagulative necrosis were also present. The tumor cells were positive for vimentin, HMB45, and Melan-A, and focally positive for SMA and S-100 protein. After 5 years and 5.6 years of nephrectomy, the tumor metastasized to the right lung and ileum, respectively.
We first reported one case of malignant PEComa of the kidney with pulmonary and ileum metastases. Metastatic PEComa of the lung and ileum should differentiate from primary carcinoma, metastatic carcinoma, malignant melanoma, and gastrointestinal stromal tumor.
报告1例肾恶性血管周上皮样细胞瘤（PEComa）伴罕见肺和回肠转移病例，并分析其临床病理特征。
分析1例肾恶性PEComa伴肺和回肠转移病例的临床病理特征。进行免疫组织化学染色。
患者为48岁男性，肾脏肿块大小约为14 cm×11 cm×8 cm。显微镜下，肿瘤主要由多边形上皮样细胞组成，胞质嗜酸性致密，细胞核圆形，核仁小。局灶性肿瘤细胞表现为多形性，可见多核巨细胞和明显核仁。肿瘤细胞巢被厚壁不规则血管环绕。肿瘤内可见局灶性脂肪细胞。还存在出血和凝固性坏死。肿瘤细胞波形蛋白、HMB45和Melan-A呈阳性，平滑肌肌动蛋白（SMA）和S-100蛋白局灶性阳性。肾切除术后5年和5.6年，肿瘤分别转移至右肺和回肠。
我们首次报告1例肾恶性PEComa伴肺和回肠转移病例。肺和回肠转移性PEComa应与原发性癌、转移性癌、恶性黑色素瘤和胃肠道间质瘤相鉴别。
