转移性透明细胞性肾细胞癌42例临床病理分析

Metastatic clear cell renal cell carcinoma: a clinicopathological analysis of 42 cases

目的 探讨转移性透明细胞性肾细胞癌（clear cell renal cell carcinoma,CCRCC）的临床病理学特征、诊断及鉴别诊断。方法 回顾性分析42例转移性CCRCC的临床病理资料,并复习相关文献。结果 42例转移性CCRCC中,男性35例,女性7例;转移部位依次为肺19例,骨8例,淋巴结及软组织各3例,肾上腺、皮肤及脑各2例,肝、小肠及鼻腔各1例。42例转移性CCRCC中,31例于肾原发灶手术切除后3周-11年出现转移,4例原发灶与转移灶同时发现;28例转移灶为单发,14例为多发性。34例转移性CCRCC仍具有原发性CCRCC的组织学形态,4例出现多灶状坏死,6例呈肉瘤样分化。Fuhrman细胞核分级：Ⅰ级4例、Ⅱ级13例、Ⅲ级18例、Ⅳ级7例。17例间质小血管呈簇状增生。免疫组化标记42例转移性CCRCC中CD10、vimentin、CAⅨ、PAX8、RCC和EMA阳性例数分别为39例（92.9%）、40例（95.2%）、38例（90.5%）、38例（90.5%）、31例（73.8%）和38例（90.5%）。结论 转移性CCRCC具有独特的临床病理学特征,结合临床病史及肿瘤组织形态学特点,联合免疫组化标记vimentin、EMA、CD10、CAⅨ、PAX8等有助于诊断及鉴别诊断。

Purpose To study the clinicopathological features, the essentials of diagnosis and differential diagnosis of metastatic clear cell renal cell carcinoma （CCRCC）. Methods The clinieopathological data of 42 cases metastatic CCRCC were collected, and immunohistochemical method was utilized to uncover the clinical features, morphologic characteristics, diagnosis and differential diagnosis of metastatic CCRCC. Results The cohort comprised of 35 males and 7 females. Among the 42 cases metastatic CCRCC, 19 cases metastasized to the lung, 8 cases metastasized to the bone. Other metastatic loci were the lymph node and soft tissue （3 cases）, adrenal gland, skin and brain （2 cases）, liver, small intestine and nasal cavity （ 1 case）, respectively. As for the metastatic time, 31 cases appeared distant metastasis between 3 weeks to 11 years after radical nephreetomy, and 4 cases presented primary carcinoma accompanied with metastasis loci at the same time. Among the 42 cases, 28 cases metastasized to single organ, and 14 cases metastasized to multiple organs. 34 cases of the metastatic CCRCC tissues still showed the same histological morphology as the primary CCRCC, 4 cases presented focal necrosis, and 6 cases presented sarcomatoid differentiation. As for Fuhrman nuclear grade, 4 cases were I grade, 13 cases were II grade, 18 cases were III grade, and 7 cases were IV grade. Additionally, 17 cases showed interstitial capillaries clustered hyperplasia. Immunohistochemically, CD10, vimentin, CAIX, PAX8, RCC and EMA protein were expressed in the metastatic loci in different degrees with the expression rates of 39（92. 9% ） , 40（95.2% ） , 38（90. 5% ） , 38（90. 5% ） , 31 （73.8%） and 38 （90. 5% ）, respectively. Conclusion Metastatic CCRCC presents unique clinicopathological features. Combing the clinical history and morphology characteristics, as well as immunohistochemical markers including vimentin, EMA, CD10, CAIX and PAX8 would fa- cilitate the diagnosis and differential diagnosis of the metastatic CCRCC.