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嗜酸性肾上腺皮质肿瘤:16例病理特征及文献复习

Oncocytic Adreno Cortical Tumors: Pathological Features of 16 Cases and Review of the Literature.

作者信息

Ertan Yesim, Argon Asuman, Özdemir Murat, Yürekli Banu Pınar Sarer, Dökümcü Zafer, Makay Özer

机构信息

Department of Pathology, Ege University, Izmir, Turkey.

Department of Pathology, Izmir Bozyaka Training and Research Hospital, Izmir, Turkey.

出版信息

J Environ Pathol Toxicol Oncol. 2017;36(3):237-244. doi: 10.1615/JEnvironPatholToxicolOncol.2017021895.

Abstract

Oncocytic neoplasms of the adrenal gland are extremely rare tumors. These tumors differ from their nononcocytic counterparts in some respects. The aim of this study was to review and discuss the clinical, histological, and immunohistochemical features of as well as the prognosis for these rare tumors. In total, 16 cases diagnosed as adrenocortical oncocytic neoplasms between January 2011 and December 2016 were included in the study. The demographic data, gross characteristics, histological data, and immunohistochemical data (Chromogranin-A, Synaptophysin, α-Inhibin, Melan-A, Ki67, PHH3) were reevaluated. The follow-up data for these patients were added in January 2017. Of the 16 cases, 12 were adrenocortical adenoma, 1 was borderline adrenocortical tumor, and 3 were adrenocortical carcinoma. The tumors equally affected both genders. The tumors were not generally large. Tumor cells had pleomorphic nuclei in ten cases, but it was more obvious in one case. The mitotic figure count was low in most tumors. Atypical mitosis and necrosis were observed in three and four tumors, respectively. None of cases included sinusoidal invasion, vascular invasion, or capsular invasion. We detected the expression of at least one specific marker (e.g., Melan-A, Inhibin-α) of the adrenal cortex in all tumors. None of the tumors were immunoreactive for Chromogranin-A. Ki-67 proliferation index was lower than 5% in all cases except three oncocytic carcinomas. In two cases, PHH3 positivity was not seen, while it was lower than 3 of 10 high-powered fields in ten cases and higher in 4 cases. All patients were alive and disease free except for two patients with adrenocortical carcinoma. In conclusion, determining the clinical, histological, and immunohistochemical characteristics of these extremely rare tumors can provide important information for early diagnosis, treatment, and follow-up of these cases.

摘要

肾上腺嗜酸性细胞瘤是极其罕见的肿瘤。这些肿瘤在某些方面与其非嗜酸性对应物有所不同。本研究的目的是回顾和讨论这些罕见肿瘤的临床、组织学和免疫组化特征以及预后。本研究共纳入了2011年1月至2016年12月期间诊断为肾上腺皮质嗜酸性细胞瘤的16例病例。重新评估了人口统计学数据、大体特征、组织学数据和免疫组化数据(嗜铬粒蛋白A、突触素、α-抑制素、黑色素A、Ki67、磷酸化组蛋白H3)。2017年1月补充了这些患者的随访数据。16例病例中,12例为肾上腺皮质腺瘤,1例为临界性肾上腺皮质肿瘤,3例为肾上腺皮质癌。肿瘤在两性中发病率相同。肿瘤一般不大。10例肿瘤细胞有异形核,但1例更明显。大多数肿瘤的分裂象计数较低。分别在3例和4例肿瘤中观察到非典型有丝分裂和坏死。所有病例均未出现窦状隙浸润、血管浸润或包膜浸润。我们在所有肿瘤中均检测到肾上腺皮质至少一种特异性标志物(如黑色素A、抑制素-α)的表达。所有肿瘤对嗜铬粒蛋白A均无免疫反应。除3例嗜酸性癌外,所有病例的Ki-67增殖指数均低于5%。2例未见到磷酸化组蛋白H3阳性,10例中有10例低于10个高倍视野中的3个,4例较高。除2例肾上腺皮质癌患者外,所有患者均存活且无疾病。总之,确定这些极其罕见肿瘤的临床、组织学和免疫组化特征可为这些病例的早期诊断、治疗和随访提供重要信息。

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