Xia Qiu-Yuan, Rao Qiu, Shen Qin, Shi Shan-Shan, Li Li, Liu Biao, Zhang Jin, Wang Yan-Fen, Shi Qun-Li, Wang Jian-Dong, Ma Heng-Hui, Lu Zhen-Feng, Yu Bo, Zhang Ru-Song, Zhou Xiao-Jun
Department of Pathology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
Int J Clin Exp Pathol. 2013 Jun 15;6(7):1392-9. Print 2013.
Papillary renal cell carcinoma (PRCC) is traditionally classified into type 1 and type 2. Recently, an oncocytic variant of PRCC has been described. We report a series of 6 oncocytic renal papillary tumors (OPRCC) which tended to occur in older patients (mean, 56.8 years) with a male preference (male-to-female ratio is 5:1). All 6 patients are alive with no evidence of disease after initial resection, showing an indolent clinical behavior. Histologically, tumors exhibited predominant papillary structure with delicate fibrovascular cores. Papillae were lined by single layers of cells with large, deeply eosinophilic and finely granular cytoplasms and round regular nucleus. The phagocytosis of tumor cells was frequently and evidently seen in our cases that hemosiderin-laden tumor cells and foamy tumor cells were noticed in five and four cases respectively. All tumors were immunoreactive for racemase, vimentin, CD10, and MET and negative for CD117. While E-cadherin, EMA, and cytokeratin 7 exhibited variable immunopositivity. FISH analysis was performed in five of six cases and found heterogeneous results. Trisomy of chromosomes 7 was found in three cases and trisomy of chromosomes 17 in two cases. Loss of chromosome Y was noted in one of four tumors in male patients. MET gene status was also investigated by direct sequencing in all 6 cases and found no distinct mutation in any case. These results suggest that OPRCC shows distinct morphology, indolent clinical behavior, and similar immunohistochemical and cytogenetic features with PRCC, seems to be a variant in the PRCC group. Whether the strong expression of MET indicates a potential therapeutic target is still unknown and requires further investigation in clinical trials.
乳头状肾细胞癌(PRCC)传统上分为1型和2型。最近,已描述了PRCC的一种嗜酸细胞变体。我们报告了一系列6例嗜酸细胞性肾乳头状肿瘤(OPRCC),这些肿瘤倾向于发生在老年患者(平均56.8岁)中,男性居多(男女比例为5:1)。所有6例患者在初次切除后均存活,无疾病证据,显示出惰性的临床行为。组织学上,肿瘤表现为以纤细纤维血管轴心为主的乳头状结构。乳头由单层细胞衬里,这些细胞具有大的、嗜酸性深且颗粒细腻的细胞质以及圆形规则的细胞核。在我们的病例中经常明显可见肿瘤细胞的吞噬作用,分别在5例和4例中观察到含铁血黄素丰富的肿瘤细胞和泡沫状肿瘤细胞。所有肿瘤对消旋酶、波形蛋白、CD10和MET均呈免疫反应性,对CD117呈阴性。而E-钙黏蛋白、EMA和细胞角蛋白7表现出可变的免疫阳性。对6例中的5例进行了荧光原位杂交(FISH)分析,结果各异。3例发现7号染色体三体,2例发现17号染色体三体。在男性患者的4例肿瘤中有1例发现Y染色体缺失。还对所有6例进行了直接测序以研究MET基因状态,未发现任何病例有明显突变。这些结果表明,OPRCC显示出与PRCC不同的形态、惰性的临床行为以及相似的免疫组化和细胞遗传学特征,似乎是PRCC组中的一个变体。MET的强表达是否表明一个潜在的治疗靶点仍不清楚,需要在临床试验中进一步研究。