原发性肾脏散发性血管母细胞瘤1例报告及文献复习

Jun Zhang, Ning Wang, Li-hong Chen, Wenjuan Wang, Ming Wang, Hao Liu, Han-guo Jiang, Y. Qi
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引用次数: 1

摘要

散发性肾血管母细胞瘤是一种罕见的神经外血管母细胞瘤亚群。它们是未被充分认识的肾肿瘤,其鉴别诊断仍然具有挑战性。在这里,我们描述一个病例RHB在一个61岁的男子入院两天后发现一个右肾肿块。临床诊断为肾癌,行根治性肾切除术。随访未见术后肿瘤复发。组织学上,肿瘤边界清晰,可见纤维包膜。肿瘤问题主要由肿瘤细胞和树突状毛细血管网络组成,该网络由多细胞和少细胞区域组成。肿瘤细胞呈多边形,细胞质嗜酸性或透明,核内可见假包涵体。免疫组织化学结果显示,vimentin、a-抑制素、神经源性特异性烯醇化酶(NSE)、S-100、平滑肌肌动蛋白(SMA)和分化簇(CD)10抗体反应强烈,呈弥散状,Ki-67阳性2%。CD31和CD34表现为血管形态。我们还总结了相关病例报告(共41例中英文文献),以探讨RHB的临床病理特征,提高本病的诊断和治疗水平。RHB是一种预后良好的良性肿瘤;然而,它很容易被误诊为其他常见的肾脏恶性肿瘤。免疫组织化学对RHB的准确诊断有很大的帮助。术前肾活检可有效避免误诊和过度治疗。
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Primary renal sporadic hemangioblastoma: A case report and literature review
Sporadic renal hemangioblastomas (RHBs) are a rare subgroup of extraneurologic hemangioblastomas. They are under-recognized renal tumours whose differential diagnosis remains challenging. Here, we describe a case of RHB in a 61-year-old man was admitted to the hospital two days after the discovery of a right kidney mass. Renal carcinoma was clinically considered, and a radical nephrectomy was performed. Follow-up showed no evidence of postoperative tumour recurrence. Histologically, the tumour boundary is clear and fibrous envelope is visible. The tumour issue was mainly composed of tumour cells and a dendritic capillary network, which consisted of multicellular and oligocellular areas. The tumour cells were polygonal, the cytoplasm was eosinophilic or transparent, and intranuclear pseudoinclusions were found. Immunohistochemically, vimentin, a-inhibin, neurogenic specific enolase (NSE), S-100, smooth muscle actin (SMA), and cluster of differentiation (CD)10 antibodies reacted strongly and were diffused, and Ki-67 was 2% positive. CD31 and CD34 showed vascular morphology. We also summarized related case reports (a total of 41 cases in the Chinese and English literature) to explore the clinicopathological characteristics of RHB and improve the diagnosis and treatment of this disease. RHB is a benign tumour with excellent prognosis; however, it is easily misdiagnosed as other common renal malignancies. Immunohistochemistry is vastly helpful in accurate diagnosis of RHB. Preoperative renal biopsy can effectively avoid misdiagnosis and overtreatment.
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