Acquired cystic disease-associated renal cell carcinoma (ACD-RCC) is the major RCC subtype in patients with end-stage renal disease, specifically those with ACD on dialysis. Three patients with a total of eight tumors have been selected. The aim of this study was to analyze clinicopathologic, immunohistochemical, and prognostic features of eight ACD-RCCs. Three patients with end-stage renal disease (ESRD) were in the age range of 34–45 years and being treated with hemodialysis. All eight tumors were resected by radical nephrectomy. Two patients had a single ACD-RCC, while one patient had bilateral and multifocal ACD-RCCs. Microscopically, combinations of architectural patterns were identified in all tumors. Intracytoplasmic and intraluminal vacuoles, eosinophilic granular cytoplasm, and prominent nucleoli were universal characteristics of these tumors. Atypical cysts were present in three out of four resected kidneys. Immunohistochemistry (IHC) staining revealed all tumors were strongly and diffusely positive for pan-cytokeratin and α-methylacyl-CoA racemase and variably positive for CK7, CD10, PAX8, EMA, vimentin, cytokeratin, high molecular weight cytokeratin (CK HMW). All cases were negative for Napsin A, CK20, CD117, and CD57. After an average follow-up of 27.5 months (range 3–54 months), all our patients are alive without neoplastic (metastatic or recurrent) disease. Our study supports the finding that ACD-RCC has specific morphologic features and a broad spectrum of architectural patterns. We have found that the immunoprofile of ACD-RCC is distinct from that in other RCCs; however, nonspecific and interpretation of microscopic features in the context of the clinical history can aid the diagnosis. We confirm also the favorable prognosis in ACD-RCC.


中文翻译：

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获得性囊性疾病相关肾细胞癌：8 种肿瘤的临床病理学研究及临床随访


获得性囊性疾病相关肾细胞癌 (ACD-RCC) 是终末期肾病患者的主要 RCC 亚型，特别是那些接受透析的 ACD 患者。已经选择了总共有八个肿瘤的三名患者。本研究的目的是分析八种 ACD-RCC 的临床病理学、免疫组化和预后特征。三名终末期肾病（ESRD）患者年龄在 34-45 岁之间，正在接受血液透析治疗。所有八个肿瘤均通过根治性肾切除术切除。两名患者患有单个 ACD-RCC，而一名患者患有双侧和多灶性 ACD-RCC。在显微镜下，在所有肿瘤中都发现了结构模式的组合。胞浆内和腔内空泡，嗜酸性粒状细胞质，和突出的核仁是这些肿瘤的普遍特征。四分之三的切除肾脏中存在非典型囊肿。免疫组织化学 (IHC) 染色显示，所有肿瘤对泛细胞角蛋白和 α-甲基酰基-CoA 消旋酶呈强烈和弥漫性阳性，并且对 CK7、CD10、PAX8、EMA、波形蛋白、细胞角蛋白、高分子量细胞角蛋白 (CK HMW) 呈可变阳性。所有病例 Napsin A、CK20、CD117 和 CD57 均为阴性。在平均随访 27.5 个月（范围 3-54 个月）后，我们所有的患者都没有出现肿瘤（转移性或复发性）疾病。我们的研究支持 ACD-RCC 具有特定的形态特征和广泛的结构模式这一发现。我们发现 ACD-RCC 的免疫特征与其他 RCC 不同；然而，非特异性和结合临床病史对微观特征的解释有助于诊断。我们还证实了 ACD-RCC 的良好预后。