Wang Yiqiu, Ding Ying, Qin Chao, Gu Min, Wang Zengjun, Han Conghui, Liu Xia, Li Hongxia, Hua Hongjin
Department of Urology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China; Department of Surgical Oncology, The Affiliated Xuzhou Hospital of Medical College of Southeast University, Xuzhou, China.
Department of Pathology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China.
Ann Diagn Pathol. 2018 Oct;36:1-4. doi: 10.1016/j.anndiagpath.2018.06.007. Epub 2018 Jun 20.
Clear cell papillary renal cell carcinoma (ccpRCC) is a recently recognized subtype of renal cell carcinoma. In this study, we investigated the clinicopathological and immunohistochemical features in a group of 26 cases of ccpRCC, with a special emphasis on the expression of vitamin D receptor (VDR). The mean age of patients was 53.3 years (range 36-74 years), and the mean tumor size was 2.5 cm (range 0.5 to 6.5 cm). During follow-up (range 12-121 months, median 50 months), no recurrence or metastasis was observed. Histopathologically, all cases of ccpRCC exhibited a tubular and papillary architecture, covered by tumor cells with clear cytoplasm. Immunohistochemistry showed intermediate (5/26, 19%) to diffuse (21/26, 81%) and moderate (2/26, 8%) to strong (24/26, 92%) membranous staining for VDR in each case. All cases (26/26, 100%) were diffuse and strong cytoplasmic and fibrillar staining for cytokeratin 7 (CK7), but negative forα-methylacyl-CoA-racemase (AMACR). Each case showed diffuse (26/26, 100%) and moderate (4/26, 15%) to strong (22/26, 85%) membranous staining for carbonic anhydrase IX (CA IX). In addition, the majority of cases showed negative for cluster of differentiation 10 (CD10) (20/26, 77%) and renal cell carcinoma maker (RCC-Ma) (24/26, 92%). This unique staining pattern is helpful for distinguishing ccpRCC from its mimics. Furthermore, VDR positive expression suggests that ccpRCC originates from the precursor epithelium of distal nephron.
透明细胞乳头状肾细胞癌（ccpRCC）是一种最近才被认识的肾细胞癌亚型。在本研究中，我们调查了一组26例ccpRCC的临床病理和免疫组化特征，特别关注维生素D受体（VDR）的表达。患者的平均年龄为53.3岁（范围36 - 74岁），平均肿瘤大小为2.5厘米（范围0.5至6.5厘米）。在随访期间（范围12 - 121个月，中位数50个月），未观察到复发或转移。组织病理学上，所有ccpRCC病例均表现为管状和乳头状结构，被细胞质透明的肿瘤细胞覆盖。免疫组化显示，每例VDR呈中等强度（5/26，19%）至弥漫性（21/26，81%）以及中度（2/26，8%）至强阳性（24/26，92%）的膜染色。所有病例（26/26，100%）细胞角蛋白7（CK7）呈弥漫性且强阳性的细胞质和纤维状染色，但α - 甲基酰基辅酶A消旋酶（AMACR）为阴性。每例碳酸酐酶IX（CA IX）呈弥漫性（26/26，100%）以及中度（4/26，15%）至强阳性（22/26，85%）的膜染色。此外，大多数病例分化簇10（CD10）（20/26，77%）和肾细胞癌标志物（RCC - Ma）（24/26，92%）呈阴性。这种独特的染色模式有助于将ccpRCC与其相似病变区分开来。此外，VDR阳性表达提示ccpRCC起源于远端肾单位的前体上皮。
