Abstract
Clear cell papillary renal cell carcinoma is increasingly recognized as a distinct tumor with unique morphology, immunohistochemistry, and cytogenetics. Histopathology often mimics clear cell renal cell carcinoma; however, metastasis has not been reported, emphasizing the clinical value of recognizing these likely nonaggressive tumors. We studied tumors with borderline morphology of clear cell papillary renal cell carcinoma, utilizing immunohistochemistry and fluorescence in situ hybridization or karyotyping. Tumors from 22 patients (ages 33 to 82 y) were analyzed. Clear cell papillary renal cell carcinoma-like morphology varied from 10% to 90% of the tumor (median 25%). Sources of resemblance included: branched glands (95%), nuclear alignment (68%), small papillary tufts (32%), focal branching papillae (27%), and prominent papillary structures (9%). Carbonic anhydrase IX uniformly revealed diffuse positivity. Staining for cytokeratin 7 (CK7) was focal (64%) or negative (18%) in most tumors (82%); however, >50% labeling was present in 4 (18%). Reactivity for both CD10 and α-methyl-acyl-CoA-racemase (AMACR) was usually present (median 80% and 60% of cells). Seven tumors showed reactivity for high-molecular weight keratin (32%). Chromosome 3p loss was confirmed in 15 tumors (68%), including 4/7 with labeling for high-molecular weight keratin or >50% reactivity for CK7. A discordant immunohistochemical pattern typically correlates with loss of material from chromosome 3p in tumors with incomplete morphology of clear cell papillary renal cell carcinoma, supporting classification as clear cell renal cell carcinoma. Diffuse labeling for CK7 can uncommonly be observed in clear cell renal cell carcinomas confirmed to have chromosome 3p loss, although these do not exhibit the expected staining pattern of clear cell papillary renal cell carcinoma, including positivity for CD10 and AMACR.
摘要
透明细胞乳头状肾细胞癌逐渐被视为一种具有独特形态学、免疫表型和细胞遗传学特点的肿瘤,其组织病理学特征常常类似透明细胞性肾细胞癌,但未见转移的报道。因此,该肿瘤可能是一种非侵袭性肿瘤,认识到这一点具有非常重要的临床意义。作者采用免疫组织化学、荧光原位杂交或染色体核型分析技术,对22例(年龄33-82岁)具有交界性形态学特点的透明细胞乳头状肾细胞癌进行分析。结果发现,22例肿瘤中,与透明细胞乳头状肾细胞相似的形态学表现所占比例从10%-90%不等(平均25%),包括:分支状腺体(95%)、核排列在基底膜上(68%)、簇状微乳头(32%)、局灶性分支乳头(27%)及明显的乳头状结构(9%)。免疫组化结果显示:碳酸酐酶IX均匀弥漫阳性;大部分病例(82%)CK7局灶阳性(64%)或阴性(18%),另外4例(18%),>50%的区域CK7阳性;CD10和α-甲酰基辅酶A消旋酶(AMACR)通常阳性(中位值为60%和80%);7例(32%)高分子量角蛋白阳性;15例(68%)(包括4/7例高分子量角蛋白阳性或CK7阳性率>50%的病例)存在染色体3p丢失。透明细胞性肾细胞癌中已证实存在染色体3p丢失,且极少CK7弥漫阳性,而上述病例并没有显示透明细胞乳头状肾细胞的着色模式,包括CD10及AMACR阳性。不一致的免疫组化模式通常与形态学不完全的透明细胞乳头状肾细胞癌肿瘤中染色体3p缺失有关,因此支持将该肿瘤归为透明细胞性肾细胞癌。
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