2018 年第 2 期 第 13 卷

甲状腺样滤泡性肾细胞癌的临床特点分析

作者：杨波胡浩耿紫娴刘士军王晓峰徐涛

英文作者：

单位：100044北京大学人民医院泌尿外科

英文单位：

关键词：甲状腺样滤泡性肾细胞癌；肾肿瘤

英文关键词：

• 摘要：

• 目的    分析甲状腺样滤泡性肾细胞癌（TLF-RCC）的临床特点。方法    对2014年11月7日北京大学人民医院收治1例及文献检索27例TLF-RCC患者的一般情况、临床症状、病变特点、免疫组织化学检测、肿瘤转移、伴随疾病、随访与转归进行综合分析。结果    28例患者中男10例（35.7%）、女18例（64.3%）、男女比例1∶1.8。年龄19～83岁，中位年龄35（30，53）岁。有临床症状者15例（53.6%），偶然发现者13例（46.4%）。临床症状包括无痛性血尿、腰痛、腹痛等，大多为慢性病程。所有TLF-RCC均为单发肿瘤，肿瘤长径为1.1～11.8 cm，中位长径4.0（2.5，6.0）cm。免疫组织化学染色结果为甲状腺球蛋白与甲状腺转录因子1均阴性，其他标志物特点为肌酸激酶（+）、CAM5.2（+）、35βH11（+）、波形蛋白（+）、细胞角蛋白7（+）、上皮细胞膜抗原（+）、细胞角蛋白19（+）与Pax8（+），α甲酰基辅酶A消旋酶（-）、CD57（-）、CD10（-）、CD117（-）与RCC（-），Ki-67大多<5%（+）。6例患者（21.4%）发生肿瘤转移，其中4例转移与TLF-RCC同时诊断，余2例为根治术后诊断；转移部位包括肺（3例），肾门淋巴结（2例）和腹腔（2例）。所有患者均接受手术治疗，随访时间为1～84个月，中位随访时间11（6，21）个月。21例患者（75.0%）无复发，1例（3.6%）靶向治疗后出现肺转移瘤，1例（3.6%）术后2个月出现肺转移，1例（3.6%）术后7个月出现腹腔淋巴结转移；1例（3.6%）死亡。结论    TLF-RCC是一种非常罕见的低度恶性肾脏肿瘤，好发于青年女性，临床表现无特异性，病情进展缓和。TLF-RCC预后较好，尚不明确肿瘤转移的危险因素。

• Clinical features of thyroid-like follicular renal cell carcinoma

  Yang Bo, Hu Hao, Geng Zixian, Liu Shijun, Wang Xiaofeng, Xu Tao

  Department of Urology, Peking University People′s Hospital, Beijing 100044, China

  Corresponding author: Xu Tao, Email: xutao@medmail.com

  【Abstract】Objective    To analyze clinical feature of thyroid-like follicular renal cell carcinoma（TLF-RCC）. Methods    General information, clinical symptoms, pathologic features, immunohistochemical results, metastases, concomitant diseases, follow-up data and prognosis of TLF-RCC were analyzed in 1 patient with TLF-RCC who were admitted on November 7th, 2014 in Peking University People′s Hospital and 27 cases of TLF-RCC retrieved from literature base. Results    There were 10 males（35.7%） and 18 females（64.3%）; the male to female ratio was 1∶1.8. The median age was 35.0（30.5, 53.0） years old（19-83 years old）. Fifteen patients（53.6%） had clinical symptoms（long course of painless hematuria, backache and choronic abdominal pain） and 13 patients（46.4%） were diagnosed accidentally. All patients had single tumor; the diameter of tumor ranged from 1.1 to 11.8 cm, and the median diameter was 4.0（2.5, 6.0）cm. Immunohistochemical results were as follows: thyroglobulin（－）, thyroid transcription factor-1（－）, creatine kinase（＋）, CAM5.2（＋）, 35βH11（＋）, vimentin（＋）, cytokeratin 7（＋）, epithelial membrane antigen（＋）, cytokeratin 19（＋）, Pax8（＋）, α-methylacyl coenzyme A racemase（－）, CD57（－）, CD10（－）, CD117（－）, RCC（－） and mostly Ki-67＜5%（＋）. Six patients（21.4%） had metastasis; 4 patients were diagnosed of TLF-RCC accompanied by metastasis, and 2 patients were discovered metastasis after nephrectomy. Metastases of tumor located on lung（3 cases）, renal hilus（2 cases） and abdominal cavity（2 cases）. All patients had surgical treatment and they were followed up for 1-84 months; the median follow-up time was 11（6, 21） months. Twenty-one patients（75.0%） had no recurrence; 1 patient（3.6%） had lung metastasis after targeted therapy; 1 patient（3.6%） had lung metastasis in 2 months after nephrectomy; 1 patient（3.6%） had celiac lymphatic metastasis in 7 months after nephrectomy; 1 patient（3.6%） died. Conclusions    TLF-RCC is a rare renal tumor with low grade malignancy; it is common in young women with no specific clinical symptoms and a chronic course. The prognosis of TLF-RCC is good in most patients; risk factors of tumor metastasis remain unclear.

  【Key words】Thyroid-like follicular renal cell carcinoma;Kidney neoplasms