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原发性肾脏高分化神经内分泌肿瘤:来自印度北部一家三级护理中心的六例病例分析,并复习文献。

Primary Renal Well-Differentiated Neuroendocrine Tumors: Analyis of Six Cases from a Tertiary Care Center in North India with Review of Literature.

机构信息

Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, India.

Department of Urology and Renal Transplantation, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, India.

出版信息

Int J Surg Pathol. 2023 Sep;31(6):982-992. doi: 10.1177/10668969221113494. Epub 2022 Jul 29.

Abstract

Well-differentiated renal neuroendocrine tumors are rare tumors. As their biologic behavior is not fully known, there is a need to know more about these cases. We performed a retrospective chart review of all the cases diagnosed with renal neuroendocrine tumors from January 2016 to December 2020 (five years) in order to understand their clinical features, morphological characteristics and outcome. We included six cases with mean age of 46.2 years (4 males) in our study. All patients underwent radical nephrectomy. Histologically all showed tumor disposed in nests and trabeculae and majority of the tumors belonged to well-differentiated neuroendocrine tumor Grade 1 (WHO criteria of gastoroenteropancreatic neuroendocrine neoplasms). Lymph node metastasis was seen in two cases at the time of clinical presentation. All the tumors were diffusely positive for neuroendocrine tumor markers (synaptophysin, chromogranin, NSE, CD56). Follow-up data was available in all cases with an average follow-up of two years and neither has shown evidence of metastasis or relapse till last follow-up. Role of morphological patterns and immunohistochemical markers is highlighted with the importance of including Ki-67 index in grading them to better understand their outcome.

摘要

分化良好的肾神经内分泌肿瘤是罕见的肿瘤。由于其生物学行为尚不完全清楚,因此需要更多地了解这些病例。我们对 2016 年 1 月至 2020 年 12 月(五年)期间诊断为肾神经内分泌肿瘤的所有病例进行了回顾性图表审查,以了解其临床特征、形态特征和结局。我们的研究纳入了 6 例平均年龄为 46.2 岁(4 例男性)的患者。所有患者均接受了根治性肾切除术。组织学上均显示肿瘤呈巢状和小梁状排列,大多数肿瘤属于分化良好的神经内分泌肿瘤 1 级(胃肠胰神经内分泌肿瘤的世界卫生组织标准)。2 例患者在临床就诊时出现淋巴结转移。所有肿瘤均弥漫性表达神经内分泌肿瘤标志物(突触素、嗜铬粒蛋白、NSE、CD56)。所有病例均获得随访数据,平均随访 2 年,末次随访均未发现转移或复发的证据。强调了形态模式和免疫组织化学标志物的作用,并强调在对其进行分级时纳入 Ki-67 指数以更好地了解其结局的重要性。

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