透明细胞乳头状肾细胞癌临床病理特征分析

Clinicopathological analysis of clear cell papillary renal cell carcinoma

目的:探讨透明细胞乳头状肾细胞癌(CCPRCC)的临床及病理学特征。方法:回顾性分析6例CCPRCC的临床特征、组织学特点及免疫表型,并复习相关文献。结果:6例肿瘤均位于肾皮质内,肿瘤边界清晰,有纤维性包膜,切面灰红或灰黄色,部分呈囊性改变。镜下肿瘤呈乳头状、管状、囊性及实性混合性生长,胞浆透明,细胞核远离基底膜,世界卫生组织(World Health Organization,WHO)/国际泌尿病理协会(International Society of Urological Pathology,ISUP)分级为1级或2级。免疫表型:6例肿瘤组织均表达CK7、PAX-8和34βE12,CAIX呈“杯状”或完全膜阳性表达。1例CD10部分阳性,其余5例CD10阴性。所有病例AMACR和TFE3均为阴性。结论:透明细胞乳头状肾细胞癌是一种少见的肾肿瘤,呈惰性生物学行为。形态上应与具有透明细胞和乳头状结构的肾细胞癌鉴别,可借助免疫组织化学和分子遗传学检测予以鉴别。

Objective:To investigate the clinicopathologic characteristics of clear cell papillary renal cell carcinoma(CCPRCC).Methods:The clinical features,histological changes and immune phenotypes of 6 cases of CCPRCC were retrospectively analyzed and relevant literatures were reviewed.Results:All tumors were located in the renal cortex,and were with a well circumscribe and fibrous encapsulate.The tumors were composed histologically of cells with clear cytoplasm arranged in papillary,tubular,cystic and solid patterns.The cells with nucleus were far away from the basal aspect.The tumors were of WHO/ISUP grade 1 or 2.Immunohistochemically,all cases exhibited moderate to strong positivity for CK7,PAX-8 and 34βE12,and showed"cup-like"or totally membrane positive for CAIX.A case of tumor expressed partly positive for CD10,while the remaining 5 cases of CCPRCC were negative for CD10.AMACR and TFE3 were negative reactions in any tumor.Conclusion:CCPRCC is a rare tumor of the kidney with the indolent clinical course.The differential diagnosis should be included tumors with clear cell and papillary structures.Immunohistochemical and molecular analysis may be helpful for differential diagnosis.