透明细胞乳头状肾细胞癌7例报告并文献复习

背景与目的：透明细胞乳头状肾细胞癌是最近发现的一种少见的肾脏上皮源性恶性肿瘤，其生物学行为惰性，预后较好，该研究旨在分析透明细胞乳头状肾细胞癌的临床及病理学特点，与其他亚型鉴别，以免过度治疗。方法：收集7例透明细胞乳头状肾细胞癌病例，应用组织病理学、免疫组织化学法并结合相关文献分析其镜下及临床特点。结果：7例患者肿块均位于肾内，切面灰红或灰黄色，实性或囊实性，镜下肿瘤组织呈腺管状、微囊状或乳头状结构，肿瘤以一种结构为主或多种结构混合存在。细胞质透明，细胞核圆形或卵圆形，世界卫生组织（World Health Organization，WHO）/国际泌尿病理协会（International Society of Urological Pathology，ISUP）细胞核分级为1或2级。免疫表型：7例均表达CK7、CK8、vimentin、PAX-8、CA9和CK34βE12，Ki-67增殖指数为5%~10%，7例均不表达CD117、TFE3和CD10。对本组7例患者随访2个月至4年，均无复发及转移。结论：透明细胞乳头状肾细胞癌是一种少见的低度恶性肿瘤，形态学上与多种具有透明细胞和（或）乳头状细胞的肾癌有重叠，需要借助免疫组织化学进行鉴别。

Seven cases of clear cell papillary renal cell carcinoma and review of literature

Background and purpose: Clear cell papillary renal cell carcinoma is a rare renal epithelial malignant tumor which was recently discovered. Its biological behavior is inert, and its prognosis is good. The purpose of this article was to analyze the clinical and pathological features of clear cell papillary renal cell carcinoma, and differentiate it from other subtypes to avoid overtreatment. Methods: Seven cases of clear cell papillary renal cell carcinoma were collected and analyzed by histopathology and immunohistochemistry. Results: In 7 cases, the masses were all located in the kidneys. The incised surface was grayish red and grayish yellow, solid or cystic. Under the microscope, the tumor tissue was glandular tubular, microcystic, or papillary. The tumor could be a dominant structure or a variety of structures mixed presence. The cytoplasm was clear, the nucleus was round or ovoid, and WHO/ISUP grading was grade 1 or 2. Immunophenotype results showed that 7 cases all expressed CK7, CK8, vimentin, PAX-8, CA9, CK34βE12, and Ki-67 proliferative index 5 % -10 %. However, none of them expressed CD117, TFE3 or CD10. All the patients were followed up for 2 months to 4 years without recurrence or metastasis. Conclusion: Clear cell papillary renal cell carcinoma is a rare low-grade malignant tumor. Morphologically, it overlaps with a variety of renal cancers with clear cells and/or papillary cells, which can be differentiated by immunohistochemistry.