伴有横纹肌肉瘤样转化的肾恶性混合上皮和间质肿瘤，表现为肉眼血尿。
Malignant mixed epithelial and stromal tumor of the kidney with rhabdomyosarcomatous transformation presenting with gross hematuria.
作者信息
Shahrokh Soroush, Abolhasani Maryam, Nayeri Reza Kaffash
机构信息
Department of Internal Medicine, University of Houston College of Medicine/HCA Houston Healthcare-Kingwood, Houston, Texas.
Department of Pathology, Hasheminejad Kidney Center, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
出版信息
Proc (Bayl Univ Med Cent). 2022 Jul 5;35(6):837-839. doi: 10.1080/08998280.2022.2090788. eCollection 2022.
Malignant rhabdomyosarcoma associated with mixed epithelial and stromal tumor of the kidney (MESTK) is an exceptionally rare clinical phenomenon, with only two reported cases. Here, we describe the case of a middle-aged Middle Eastern woman with a left renal mass diagnosed as malignant MESTK with rhabdomyosarcomatous transformation. The patient initially presented with gross hematuria and was found to have a heterogenous 6.3 × 4.0 cm left renal mass, initially suspected to be renal cell carcinoma. She underwent nephrectomy with surgical resection of the tumor. Microscopic exam revealed neoplastic tissue composed of spindle and epithelial cells. The immunohistochemical analysis revealed positive staining for desmin, SMA, PR and negative staining for EMA, HMB 45, Melan A, and ER, consistent with MESTK with malignant rhabdomyosarcomatous transformation. The patient's whole-body positron emission tomography/computed tomography revealed no residual malignancy, and the patient currently remains under clinical observation.
恶性横纹肌肉瘤合并肾混合上皮和间质瘤（MESTK）是一种极其罕见的临床现象，仅有两例报道。在此，我们描述了一名中东中年女性的病例，其左肾肿块被诊断为伴有横纹肌肉瘤转化的恶性MESTK。患者最初表现为肉眼血尿，发现左肾有一个6.3×4.0厘米的异质性肿块，最初怀疑是肾细胞癌。她接受了肾切除术及肿瘤手术切除。显微镜检查显示肿瘤组织由梭形细胞和上皮细胞组成。免疫组化分析显示结蛋白、平滑肌肌动蛋白、孕激素受体染色阳性，上皮膜抗原、HMB 45、黑素A和雌激素受体染色阴性，符合伴有恶性横纹肌肉瘤转化的MESTK。患者的全身正电子发射断层扫描/计算机断层扫描显示无残留恶性肿瘤，患者目前仍在临床观察中。
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