Steroidogenic Acute Regulatory Protein Is a Useful Marker for Sex-Cord-Stroma Tumors and Normal and Neoplastic Adrenocortical Tissue.

Maximilian Lennartz, Daniela Amezada, Doris Höflmayer, Sebastian Dwertmann Rico, Clara von Bargen, Simon Kind, Viktor Reiswich, Florian Viehweger, Florian Lutz, Veit Bertram, Christoph Fraune, Natalia Gorbokon, Sören Weidemann, Claudia Hube-Magg, Anne Menz, Ria Uhlig, Till Krech, Andrea Hinsch, Eike Burandt, Guido Sauter, Ronald Simon, Martina Kluth, Andreas H Marx, Patrick Lebok, David Dum, Sarah Minner, Frank Jacobsen, Till S Clauditz, Christian Bernreuther, Stefan Steurer
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Abstract

Context.—: Steroidogenic acute regulatory (StAR) protein is a mitochondrial transport protein with a critical regulatory role for steroid hormone production. The tissue distribution of StAR expression is limited to few human normal tissues.

Objective.—: To assess the diagnostic and prognostic value of StAR immunohistochemistry analysis.

Design.—: A tissue microarray containing 19 202 samples from 152 different tumor types and subtypes and 608 samples of 76 different normal tissue types was analyzed by immunohistochemistry.

Result.—: StAR immunostaining occurred in 198 (1.2%) of the 17 135 analyzable tumors. StAR expression was observed in 27 of 152 tumor categories, 9 of which included at least 1 strongly positive case. The highest rate of StAR positivity occurred in Leydig cell tumors of the testis and the ovary (100%), steroid cell tumors of the ovary (100%), adrenocortical carcinomas (93%) and adenomas (87%), Sertoli-Leydig cell tumors (67%) and granulosa cell tumors of the ovary (56%), as well as seminomas (7%). Nineteen other tumor entities showed-a usually weak-StAR positivity in less than 6% of cases. A comparison with preexisting Melan-A (a melanocyte antigen) data revealed that StAR was more often positive in adrenocortical neoplasms and in Leydig cell tumors while StAR (but not Melan-A) was negative in Sertoli cell tumors.

Conclusions.—: Our data provide a comprehensive overview on the patterns of StAR immunostaining in human tumors and suggest a diagnostic utility of StAR immunohistochemistry for supporting a diagnosis of Leydig cell tumors or of normal or neoplastic adrenocortical tissue.

类固醇生成急性调节蛋白是性索-瘤、正常和肿瘤性肾上腺皮质组织的有用标记物
背景类固醇生成急性调节蛋白(StAR)是一种线粒体转运蛋白,对类固醇激素的生成具有重要的调节作用。StAR 的组织分布仅限于少数人体正常组织:评估 StAR 免疫组化分析的诊断和预后价值:通过免疫组化方法分析了包含152种不同肿瘤类型和亚型的19 202个样本以及76种不同正常组织类型的608个样本的组织芯片:在 17 135 个可分析的肿瘤中,有 198 个(1.2%)出现了 StAR 免疫染色。在 152 个肿瘤类别中有 27 个观察到了 StAR 表达,其中 9 个至少有一个强阳性病例。StAR阳性率最高的是睾丸和卵巢的Leydig细胞肿瘤(100%)、卵巢的类固醇细胞肿瘤(100%)、肾上腺皮质癌(93%)和腺瘤(87%)、Sertoli-Leydig细胞肿瘤(67%)和卵巢的颗粒细胞肿瘤(56%)以及精原细胞瘤(7%)。其他 19 个肿瘤实体的 STAR 阳性率通常较低,不足 6%。与之前已有的 Melan-A(一种黑色素细胞抗原)数据进行比较后发现,肾上腺皮质肿瘤和雷迪格细胞肿瘤中的 StAR 通常呈阳性,而 Sertoli 细胞肿瘤中的 StAR(而非 Melan-A)呈阴性:我们的数据提供了人类肿瘤中 StAR 免疫染色模式的全面概述,并表明 StAR 免疫组化在辅助诊断 Leydig 细胞肿瘤或正常或肿瘤性肾上腺皮质组织方面具有诊断作用。
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