Zhang W, Zhang L X, Yang T, Zou Y W, Liu X L, Yu W J, Jiang Y X, Li Y J
Department of Pathology, 971 Hospital of PLA Navy, Qingdao 266071, China.
Department of Pathology, the Affiliated Hospital of Qingdao University, Qingdao 266003, China.
Zhonghua Bing Li Xue Za Zhi. 2022 Oct 8;51(10):981-986. doi: 10.3760/cma.j.cn112151-20220228-00133.
To investigate the clinicopathological characteristics, immunophenotype, and molecular signatures of oncocytic papillary renal cell carcinoma (OPRCC), and to compare these findings with those in type 1 papillary renal cell carcinoma (PRCC 1). The clinicopathologic data of 19 patients with OPRCC from the Affiliated Hospital of Qingdao University (16 patients) and the 971 Hospital of People's Liberation Army Navy (3 patients) from October 2003 to February 2021 were collected. Histologic, immunohistochemical (IHC) and molecular analyses, together with a control group of 15 cases of PRCC I diagnosed in the same period, were assessed. The cohort included 15 males and 4 females, with a median age of 61 years (range, 47-78 years). In 13 patients the tumors were found at physical examination; four presented with painless gross hematuria and two with low back pain. As for the pathologic stage, 14 patients were pT1, one patient was pT2a, three patients were pT3a and one patient was pT4. The tumor size ranged from 1.7-14.0 cm, with clear boundary and soft texture. The cut surface was grayish-yellow and grayish-red. Microscopically, the tumor cells were mainly arranged in papillary (10%-100%) and acinar (tubular) patterns, with strongly eosinophilic cytoplasm, round or irregular nuclei, and prominent nucleoli (WHO/ISUP grade Ⅲ). Two cases showed sarcomatoid differentiation. Stromal foamy macrophages were visible in all cases. IHC staining showed diffuse strong positivity for AMACR in all cases. RCC (18/19), CD10 (17/19), vimentin (16/19) and PAX8 (17/19) were positive in most tumors. CK7 was expressed in about 50% of cases. Fluorescence in situ hybridization identified trisomy 7 in eight patients, trisomy 17 in seven patients, and the two aberrations occurred simultaneously in seven cases. Eight of 13 men had Y chromosome deletion. All patients were followed up for 8-120 months. Three patients died of metastases at 8, 62 and 82 months postoperatively, respectively, and one patient relapsed 36 months after surgery. Compared with PRCC1, OPRCC tended to have higher nuclear grade, and stromal foam cell aggregation was more commonly found (<0.05). The expression of CD10 and EMA were different (<0.01). There was no significant difference in the survival rate between the two groups (=0.239). OPRCC has unique morphologic features, and its immunophenotype overlaps but differs from PRCC1. The molecular results support that it belongs to a morphologic variation of PRCC. This tumor has similar biologic behavior to PRCC1, and has a poor prognosis when sarcomatoid differentiation occurs.
探讨嗜酸细胞性乳头状肾细胞癌（OPRCC）的临床病理特征、免疫表型和分子特征，并将这些结果与1型乳头状肾细胞癌（PRCC 1）进行比较。收集了2003年10月至2021年2月期间青岛大学附属医院（16例）和中国人民解放军海军第九七一医院（3例）19例OPRCC患者的临床病理资料。对其进行组织学、免疫组织化学（IHC）和分子分析，并与同期诊断的15例PRCC I对照组进行评估。该队列包括15例男性和4例女性，中位年龄为61岁（范围47 - 78岁）。13例患者在体检时发现肿瘤；4例出现无痛性肉眼血尿，2例出现腰痛。病理分期方面，14例患者为pT1，1例患者为pT2a，3例患者为pT3a，1例患者为pT4。肿瘤大小为1.7 - 14.0 cm，边界清晰，质地柔软。切面呈灰黄色和灰红色。显微镜下，肿瘤细胞主要呈乳头状（10% - 100%）和腺泡状（管状）排列，细胞质嗜酸性强，细胞核圆形或不规则，核仁明显（WHO/ISUP分级Ⅲ级）。2例显示肉瘤样分化。所有病例均可见间质泡沫状巨噬细胞。IHC染色显示所有病例中AMACR均弥漫性强阳性。大多数肿瘤中RCC（18/19）、CD10（17/19）、波形蛋白（16/19）和PAX8（17/19）呈阳性。约50%的病例中CK7表达。荧光原位杂交检测发现8例患者有7号染色体三体，7例患者有17号染色体三体，7例患者同时出现这两种畸变。13例男性中有8例存在Y染色体缺失。所有患者均随访8 - 120个月。3例患者分别在术后8、62和82个月死于转移，1例患者术后36个月复发。与PRCC1相比，OPRCC的核分级往往更高，更常见间质泡沫细胞聚集（<0.05）。CD10和EMA的表达不同（<0.01）。两组生存率无显著差异（= =0.239）。OPRCC具有独特的形态学特征，其免疫表型与PRCC1重叠但不同。分子结果支持其属于PRCC的一种形态学变异。该肿瘤与PRCC1具有相似的生物学行为，当发生肉瘤样分化时预后较差。
