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爱泼斯坦-巴尔病毒相关的肾平滑肌肿瘤:1例报告并文献复习

Epstein-Barr virus-associated renal smooth muscle neoplasm: report of a case with review of the literature.

作者信息

Creager A J, Maia D M, Funkhouser W K

机构信息

Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill School of Medicine, 27599-7525, USA.

出版信息

Arch Pathol Lab Med. 1998 Mar;122(3):277-81.

PMID:9823869
Abstract

Epstein-Barr virus (EBV) has been associated with nasopharyngeal carcinoma, some Burkitt's-type lymphomas, and posttransplantation lymphoproliferative disorder. Recently, an association between EBV and smooth muscle tumors, both malignant and benign, in the acquired immunodeficiency syndrome and posttransplantation populations has been made. We report, to our knowledge, the first case of a renal EBV-associated smooth muscle tumor. A 33-year-old man with acquired immunodeficiency syndrome presented with a mass of the left kidney that was radiographically suspicious for malignancy. He underwent left radical nephrectomy. The tumor measured 3.0 cm in the largest dimension, was well-circumscribed, and was composed of fascicles of bland spindle cells with blunt-ended nuclei, which often intersected at right angles. Focal areas of cell crowding and nuclear pleomorphism were present. No areas of lipomatous differentiation were identified. Immunohistochemically, the tumor cells were positive for desmin and muscle-specific actin and were negative for HMB-45 and CD21 (an EBV receptor). In situ hybridization with EBV-encoded RNA-1, a probe that recognizes a non-poly(A) RNA EBV transcript expressed in latently infected cells, was diffusely positive. At 6 months postnephrectomy, the patient showed no evidence of local recurrence or metastases. The incidence of this tumor is expected to increase as both the numbers of patients undergoing solid organ transplantation and the survival time of patients with the acquired immunodeficiency syndrome increase. A better understanding of the biology and pathogenesis of this entity will be important for future management of these patients.

摘要

爱泼斯坦-巴尔病毒(EBV)与鼻咽癌、某些伯基特型淋巴瘤以及移植后淋巴细胞增生性疾病有关。最近,在获得性免疫缺陷综合征患者和移植后人群中,发现EBV与恶性和良性平滑肌肿瘤之间存在关联。据我们所知,我们报告了首例与EBV相关的肾平滑肌肿瘤病例。一名患有获得性免疫缺陷综合征的33岁男性,左肾出现肿块,影像学检查怀疑为恶性肿瘤。他接受了左肾根治性切除术。肿瘤最大直径为3.0 cm,边界清晰,由具有钝端核的温和梭形细胞束组成,这些细胞束常呈直角相交。存在细胞拥挤和核多形性的局灶区域。未发现脂肪化生区域。免疫组化显示,肿瘤细胞结蛋白和肌特异性肌动蛋白呈阳性,HMB-45和CD21(一种EBV受体)呈阴性。用EBV编码的RNA-1进行原位杂交,该探针可识别潜伏感染细胞中表达的非多聚腺苷酸RNA EBV转录本,结果呈弥漫性阳性。肾切除术后6个月,患者未出现局部复发或转移迹象。随着实体器官移植患者数量的增加以及获得性免疫缺陷综合征患者存活时间的延长,预计这种肿瘤的发病率将会上升。更好地了解该实体的生物学特性和发病机制对于这些患者未来的治疗将具有重要意义。

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