苏世强^1,,
刘丽哲²,
苏晓哲¹,
陈延¹,
杨翠霞¹,
刘洪久¹,
张晋¹,
李珅^1,,
1. 石家庄市第一医院 泌尿外科, 河北 石家庄 050011
2. 河北医科大学 医学与健康研究院, 河北 石家庄 050017

基金项目: 河北省医学科学研究重点课题计划项目（20150903）；石家庄市科学技术研究与发展指导计划课题（161460743）


详细信息 作者简介: 苏世强(1980-), 男, 副主任医师。E-mail:tj777_06@163.com








通讯作者: 李珅, 主任医师。E-mail:198033111531@139.com 中图分类号: R737.11


摘要:目的探讨肾上皮样血管平滑肌脂肪瘤（epithelium angiomyolipoma，EAML）的临床病理特征及诊治情况。方法回顾性研究石家庄市第一医院2004年1月至2018年1月收治的17例肾EAML患者临床病理及预后资料，术前经超声、CT或MRI检查，7例行术中冰冻切片病理检查，术前考虑为肾血管平滑肌脂肪瘤12例、肾癌4例，肾盂肿瘤1例。17例患者均手术治疗，其中行腹腔镜下肾部分切除术10例、肾根治性切除术6例（其中腹腔镜下肾根治性切除术4例，开放肾根治性切除术2例）及腹腔镜肾输尿管全长切除术+膀胱袖状切除术1例。结果17例患者均病理诊断为肾EAML，免疫组化染色阳性率：HMB45（10/17），Melan-A（9/17）、SMA（16/17）、Ki-67 ≥ 10%（2/17）。平均随访65（8~168）个月，14例患者肿瘤无复发及转移，1例失访，2例死亡（1例患者术后36个月出现双肺转移，由于经济原因放弃治疗，术后42个月死亡；另1例患者术后12个月复查发现大网膜占位，外院手术病理提示肾上皮样血管平滑肌脂肪瘤大网膜转移，第2次术后3个月复查发现肝脏、腹膜转移，给予介入栓塞阿霉素化疗，第2次手术后6个月死亡）。结论肾EAML是一种罕见的血管平滑肌脂肪瘤亚型，确诊需要病理及免疫组化检查。治疗首选保留肾单位的手术，由于它具有潜在恶性的生物学行为，故有必要长期随访，肿瘤组织Ki-67高表达或P53突变多提示恶性可能。
关键词: 肾肿瘤/
上皮样血管平滑肌脂肪瘤/
临床病理/
预后


Abstract:ObjectiveTo investigate the clinicopathological features and prognosis of renal epithelioid angiomyolipoma (EAML) in order to improve the diagnosis and treatment.MethodsA retrospective study of clinicopathological features and prognosis data of 17 patients with renal EAML from Jan. 2014 to Jan. 2018 in the No.1 hospital of Shijiazhuang was performed. All patients were examined by ultrasound, CT or MRI before surgery and 7 patients received intraoperative pathology consultation for frozen sections.Pre-operative diagnosis included 12 cases of renal angiomyolipoma, 4 cases of renal cell carcinoma, and 1 case of renal pelvic tumor. All patients underwent surgery, including laparoscopic partial nephrectomy in 10 patients, radical nephrectomy in 6 patients (4 laparoscopic and 2 open surgeries) and total laparoscopic radical nephrectomy plus bladder sleeve resection in 1 patient.ResultsAll 17 patients were confirmed by pathological diagnosis of renal EAML. Positive immunohistochemical stains include HMB45 (10/17), Melan-A (9/17), SMA (16/17), and Ki-67 ≥ 10% (2/17). During a mean follow-up of 65 (8-168) months, 14 patients had no recurrence or metastasis, 1 patient was lost for follow-up, and 2 patients died. Among the deceased patients, one had bilateral lung metastasis 36 months after surgery, who gave up treatment due economic reasons and died 42 months after surgery. The other patient was found a large omentum occupying tumor after 12 months of surgery. Surgery and pathology in an outside hospital revealed metastasis of renal EAML in the omentum. Liver and peritoneal metastases were found again 3 months after the second operation. The patient received interventional embolization with doxorubicin and died 6 months after the second surgery.ConclusionsRenal EAML is a rare type of angiomyolipoma. The diagnosis requires pathological and immunohistochemical examinations. Nephron sparing surgery is the first choice for treatment. Because of its potentially malignant biological behavior, long-term follow-up is necessary. High Ki-67 index or P53 mutation in the tumor tissue may indicate malignancy.
Keywords:renal epithelioid angiomyolipoma/
clinicopathological features diagnosis/
prognosis/

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