肾嫌色细胞癌13例临床病理分析及文献复习

Chromophobe renal cell carcinoma：clinical pathological and literature review of 13 cases

目的 探讨肾嫌色细胞癌（CRCC）的临床病理特征、免疫表型、鉴别诊断及预后.方法 回顾性分析平煤神马医疗集团总医院2014—2017年收治的13例CRCC患者的临床病理特征、组织学分型及免疫组化检测结果,对所有的病例进行随访并进行相关文献的复习.结果 13例CRCC患者中男5例,女8例;平均年龄51岁（33～72岁）,肿瘤直径1.5～8.5 cm,平均3.6 cm.6例行根治性肾切除术,7例行保留肾单位的肾部分切除术.大体：肿瘤和邻近肾组织界限清楚,呈＂推挤边界＂和假包膜,切面实性、大多灰黄、灰红,少数可见出血及微囊性;镜下：CRCC的癌细胞呈大圆形或多边形,包膜较厚,细胞界限清楚;胞质细网状或嗜酸,核周空晕常见,核不规则,有皱褶,核仁不明显,核分裂象较少,部分可见核沟和（或）核内包涵体;间质稀少,癌细胞多数呈实性片状、巢团状、条索状排列为主,部分呈灶状的管状和小梁状排列,肿瘤中的血管大多数是厚壁血管,并伴偏心性透明变性.免疫表型：肿瘤全部表达CK（AE1/AE3）、CK7、EMA,部分表达P504s、CD117,少数表达CD10、vimentin、CA9,均不表达RCC及TFE3,13例均随访,随访时间3～32个月（平均14个月）,13例患者均健在.结论 嫌色肾细胞癌是一种少见的肾细胞癌,很少转移及复发,恶性程度相对较低,预后较好,具有典型的组织学特征,需与透明细胞癌及嗜酸性腺瘤鉴别,结合免疫表型可协助诊断,手术是其首选的治疗手段.

Objective To investigate the clinicpathologic features, immunophenotyping, differential diagnosis and prognosis of chromophobe renal cell carcinoma （CRCC）. Methods The clinico- pathological features, histological classification and immunophenotyping tests of 13 cases of CRCC admitted in General Hospital of Pingdingshan Coal Group from 2014 to 2017 were retrospectively analyzed. All of CRCC patients were followed up. The related literatures were reviewed. Results The 13 patients included 5 males and 8 females, with an average age of 51 years （33 to 72 years）. The average diameter of tumors was 3.6 cm （range from 1.5 to 8.5 cm）. Six of the 13 patients underwent radical nephrectomy, 7 patients experienced nephron-sparing suregery. Macyoscopy： the tumors and adjacent renal tissues had clear boundaries with ＂pushing boundaries＂ and pseudocapsules, cut surface was solid, mostly grayish yellow, grayish red, with few visible hemorrhages and microcystic features. In general： the tumor ceils were typically arranger in a solid sheet-like pattern. Other patterns included small nets, tubular, microcystic, trabecular. Under scope： the classic tumor showed larger pale cells with reticular cytoplasm and prominent cell membranes （plant cell-like）. The eosinophilic variant showed predominance of smaller cells. The nuclei often had a distinctive irregular wrinkled appearance with coarse ehromation, common, binucleation, and perinuclear holos, hnmunohistochemically, the tumors were all expressed CK （AE1/AE3）, CK7, EMA, partial expression of P504s, CD117, minority expression of CD10, vimentin, CA9, and none of them expressed RCC and TFE3. Thirteen patients were followed up for 3 to 32 months （14 months on average）. All of the patients survived without tumor recurrence. Conclusions Chromophobe renal cell carcino- ma is a rare subtype of renal cell carcinoma with little metastases and recurrences, CRCC is a kind of renal cell carcinoma with low malignancy and comparatively good prognosis, with typical histological features, and can be misdiagnosed as clear cell renal carcinoma, oncocytoma or other tumors. The combination of immune phenotype can help the diagnosis, surgery is the first choice of treatment.