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[低级别嗜酸性肾肿瘤(CD117阴性,细胞角蛋白7阳性)的临床病理特征:7例报告]

[Clinicopathological features of low-grade oncocytic renal tumor (CD117-negative, cytokeratin 7-positive): report of seven cases].

作者信息

Xie B, Cheng L C, Yin G L, Liu B A, Hu Z L, Tong K

机构信息

Department of Pathology, Xiangya Hospital, Central South University, Changsha 410078, China.

Department of Pathology, Bishan Hospital of Chongqing Medical University, Chongqing 402760, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2022 Aug 8;51(8):719-725. doi: 10.3760/cma.j.cn112151-20220410-00263.

Abstract

To explore clinicopathological features of low-grade oncocytic tumor (LOT) of the kidney and to analyze its relationship to hybrid oncocytic/chromophobe tumor (HOCT) of the kidney, renal oncocytoma (RO), and chromophobe renal cell carcinoma (chRCC). Seven LOTs were identified from the pathologic archives of two hospitals, including Xiangya Hospital (5 cases) and the Second Xiangya Hospital (2 cases) of Central South University between 2012 and 2019. Clinical data of the LOTs were collected. The tumor morphology was analyzed and immunohistochemistry was performed. All LOTs occurred in adults, aged from 49 to 72 years (median 56.0 years, mean 60.7 years). The tumor size ranged from 2.5 to 6.0 cm (median 4.3 cm, mean 4.3 cm). There were three male and four female patients. Three cases occurred in the left kidney and four in the right. All the tumors were solitary lesions without the clinicopathologic background of Birt-Hogg-Dubé (BHD) syndrome or oncocytosis. Five patients had available follow-up data (follow-up period 23-95 months, median 69.0 months, mean 64.6 months) and all were alive without disease. Microscopically, all LOTs were well-circumscribed (7/7). Three LOTs were partly encapsulated. The tumors demonstrated a predominant growth pattern comprising prominently compact small nests surrounded by delicately branching thin-walled blood vessels, imparting an organoid architecture (7/7), but variable numbers of glandular or gland-like structures were often seen among the small nests (7/7). There were frequently areas with loose, edematous stroma, and the tumor cells exhibited reticular, trabecular, or single cell arrangements (6/7). Focal hemorrhage was also commonly present in both compact and loose areas (5/7). In addition, focally cystic formation and ossification occurred in the compact area of one case and in the loose area of another case. The tumor cells in LOT showed intermediate cytologic characteristics between RO and chRCC, including abundantly eosinophilic granular cytoplasm, ovoid to round nuclei with mostly smooth contours, discernable small nucleoli (RO features), frequently delicate perinuclear halos, and occasional binucleation (chRCC features). The tumors were typically CK7-positive and CD117-negative (7/7), and variable staining for PAX8 (5/7), P504s (2/7), and vimentin (1/7). They were negative for CK20, CD10 and FOXI1. All tumors retained SDHB immunostaining. LOT is a rare and indolent oncocytic renal tumor with homogeneously intermediate cytologic features between RO and chRCC. There are some clinicopathologic overlaps between LOT and sporadic HOCT. The distinctive morphology and immunophenotype of LOT suggest that it is potentially a distinct tumor entity.

摘要

探讨肾低度嗜酸细胞瘤(LOT)的临床病理特征,并分析其与肾嗜酸细胞/嫌色细胞混合瘤(HOCT)、肾嗜酸细胞瘤(RO)及嫌色肾细胞癌(chRCC)的关系。从两家医院的病理档案中识别出7例LOT,包括中南大学湘雅医院(5例)和中南大学湘雅二医院(2例),时间跨度为2012年至2019年。收集LOT的临床资料。分析肿瘤形态并进行免疫组织化学检测。所有LOT均发生于成年人,年龄49至72岁(中位年龄56.0岁,平均年龄60.7岁)。肿瘤大小为2.5至6.0 cm(中位大小4.3 cm,平均大小4.3 cm)。男性3例,女性4例。3例发生于左肾,4例发生于右肾。所有肿瘤均为孤立性病变,无Birt-Hogg-Dubé(BHD)综合征或嗜酸细胞增多症的临床病理背景。5例患者有可用的随访数据(随访期23 - 95个月,中位随访期69.0个月,平均随访期64.6个月),所有患者均存活且无疾病复发。显微镜下,所有LOT边界清晰(7/7)。3例部分被膜包裹。肿瘤主要生长方式为显著紧密的小巢状结构,周围有纤细分支的薄壁血管,形成器官样结构(7/7),但小巢内常可见数量不等的腺管或腺管样结构(7/7)。常有疏松、水肿的间质区域,肿瘤细胞呈网状、小梁状或单细胞排列(6/7)。在紧密和疏松区域均常见局灶性出血(5/7)。此外,1例的紧密区域和另1例的疏松区域分别出现局灶性囊性形成和骨化。LOT中的肿瘤细胞显示出介于RO和chRCC之间的中间细胞学特征,包括丰富的嗜酸性颗粒细胞质、卵圆形至圆形核,轮廓大多光滑,可见小核仁(RO特征),常可见纤细的核周晕,偶尔双核(chRCC特征)。肿瘤通常CK7阳性,CD117阴性(7/7),PAX8(5/7)、P504s(2/7)和波形蛋白(1/7)染色可变。CK20、CD10和FOXI1均为阴性。所有肿瘤均保留SDHB免疫染色。LOT是一种罕见的惰性嗜酸细胞性肾肿瘤,在细胞学特征上均匀地介于RO和chRCC之间。LOT与散发性HOCT之间存在一些临床病理重叠。LOT独特的形态和免疫表型提示它可能是一种独特的肿瘤实体。

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