儿童肾癌的临床及CT和病理特点分析

目的 探讨儿童肾癌的临床、CT及病理特点,积累其临床诊治经验.方法 回顾性分析2014年至2016年单中心收治的7例儿童肾癌患儿的临床、CT、病理资料,并随访其生存情况.就诊时年龄5～12岁,女4例,男3例,左侧5例,右侧2例.临床表现多为血尿(4/7)、腹部包块(3/7)、腰痛(1/7).CT平扫肿瘤呈低密度,多位于上下极,可见钙化(6/7)及坏死区,增强特点多为“快进快出”(4/7),部分为“快进慢出”(2/7).结果 7例患儿全部获得随访,随访时间8～35个月.1例肿瘤位于左腹膜后,确诊时已发生腹膜后及颈部淋巴结转移,行肿瘤部分切除活检,术后5个月死亡.1例为复发性肾癌,行肾窝肿瘤切除加腹膜后淋巴结清扫,术后5个月出现淋巴结转移,予放化疗后带瘤生存.余5例患儿均行根治性肾切除加淋巴结清扫,术后均无瘤生存.病理多为肾透明细胞(5/7)及乳头状肾细胞癌(2/7),癌细胞呈巢团状、乳头状、腺管样结构分布,可见砂粒体(6/7),核级多为3～4级.4例患儿淋巴结转移,共清扫出60枚淋巴结,淋巴结阳性率为16.7％.5例加做免疫组化,TFE3 (4/5)、CD10 (5/5)、Kb67 (4/5)、E-cardherin (4/5)、Vinentin (3/5)、CK(3/5)阳性率较高,4例确诊为MiT家族异位性肾癌.结论 儿童肾癌多发生于年长患儿,有其特有的临床、CT及病理特点,MiT家族异位性肾癌常见,病理及免疫组化是确诊和分型的主要手段.

Clinical,computed tomography and pathologic features of pediatric renal cell carcinoma

Objective To explore the clinical,computerized tomography (CT) and pathologic features of pediatric renal cell carcinoma (RCC) and summarize diagnostic and treatment experiences.Methods The clinical,CT and pathologic data of 7 pediatric RCC patients at a single center during 2014-2016 were collected,compared and followed-up.There were 4 girls and 3 boys with a diagnostic age of 5-12 years.The involved side was left (n =5) and right (n =2).Common symptoms included hematuresis (4/7),abdominal mass (3/7) and lumbago (1/7).Tumor was low-density on CT plain scan with calcification (6/7) and putrescence in upper or lower pole of kidney.There was a pattern of "fast-in and fast-out" (4/7) or "fast-in and slow-out" (2/7) during enhanced scanning.Results All patients were successfully followed-up for a period of 8-35 months.One retroperitoneal RCC with retroperitoneal and cervical lymphatic metastasis at the diagnosis underwent partial tumorectomy and died at 5 months post-operation.Another recurrent RCC with retroperitoneal lymphatic metastasis underwent tumorectomy and retroperitoneal lymph node dissection.There was lymphatic metastasis at 5 months post-operation.Chemoradiotherapy was offered and the patient survived with tumor.Five primary cases underwent radical nephrectomy and retroperitoneal lymph node dissection and survived without recurrence or metastasis.Pathological types were clear (5/7) or papillary (2/7) renal cell carcinoma.Cancer cells were grown with nest clumps,papillary or glandular shapes.Calcified bodies were found in 4 cases.Nuclear grade of cancer cells was 3-4.Lymphatic metastasis was detected in 4 cases and 60 lymph nodes had a positive dissection rate of 18.6％.TFE3 (4/5),CD10 (5/5),Kk67 (4/5),E-cardherin (4/5),Vinentin (3/5),CK (3/5) were positive in 5 cases with immunostaining.Four cases were diagnosed with RCC associated with MiT family translocation.Conclusions Occurring mostly in elderly children,pediatric RCC has its uniqueness in clinical manifestation,CT imaging and pathology.RCC associated with MiT family translocation is common.Pathological and irnmunostaining examinations are essential for diagnosing definitely and typing.