CAJ | 학술논문

目的探讨肾上皮样血管平滑肌脂肪瘤的临床及病理特点.方法肾上皮样血管平滑肌脂肪瘤患者6例.单发4例,多发2例.肿瘤直径平均9(5～14)cm.有结节硬化家族史1例.CT检查发现肿瘤内含脂肪组织(CT值-30～-120 HU)3例,诊断为肾血管平滑肌脂肪瘤;3例瘤组织CT值30～55 HU,增强扫描CT值70～110 HU.行肿瘤剜除术3例,行根治性肾切除术3例.结果 3例行肿瘤剜除术者肿瘤完整剜除,2例出现肾盂破损,行肾盂修补及留置双J管.其中1例双肾肿瘤者,2个月后手术剜除对侧肿瘤.3例根治性肾切除者完整切除肾脏、肾周脂肪组织及肿大淋巴结.病理检查:镜下肿瘤以上皮样细胞为主,并出现多形、异形核,有明显的上皮样分化,浸润破坏性生长,瘤组织广泛坏死.免疫组化染色HMB45(+++),EMA、CK(-).病理诊断均为肾上皮样血管平滑肌脂肪瘤.平均随访26(10～44)个月,肿瘤无复发及转移5例;1例根治术后18个月出现双肺转移,10个月后死亡.结论肾上皮样血管平滑肌脂肪瘤多为良性,部分缺乏脂肪组织,主要由单核或多核的上皮样细胞构成,免疫组化染色HMB45强阳性为特征表现,具有潜在恶性倾向,可发生转移;CT检查易误诊为肾癌;手术为主要治疗方法.
목적 탐토신상피양혈관평활기지방류적림상급병리특점.방법 신상피양혈관평활기지방류환자6례.단발4례,다발2례.종류직경평균9(5～14)cm.유결절경화가족사1례.CT검사발현종류내함지방조직(CT치-30～-120 HU)3례,진단위신혈관평활기지방류;3례류조직CT치30～55 HU,증강소묘CT치70～110 HU.행종류완제술3례,행근치성신절제술3례.결과 3례행종류완제술자종류완정완제,2례출현신우파손,행신우수보급류치쌍J관.기중1례쌍신종류자,2개월후수술완제대측종류.3례근치성신절제자완정절제신장、신주지방조직급종대림파결.병리검사:경하종류이상피양세포위주,병출현다형、이형핵,유명현적상피양분화,침윤파배성생장,류조직엄범배사.면역조화염색HMB45(+++),EMA、CK(-).병리진단균위신상피양혈관평활기지방류.평균수방26(10～44)개월,종류무복발급전이5례;1례근치술후18개월출현쌍폐전이,10개월후사망.결론 신상피양혈관평활기지방류다위량성,부분결핍지방조직,주요유단핵혹다핵적상피양세포구성,면역조화염색HMB45강양성위특정표현,구유잠재악성경향,가발생전이;CT검사역오진위신암;수술위주요치료방법.
Objective To investigate the clinicopathologic features of epithelioid angiomyolipoma of renal(REAML). Methods Six cases of REAML were analyzed by histopathological and clinical characteristics.Of the 6 cases,4 cases were solitary tumors and 2 cases were multiple lesions.The diameter of tumors was about 9 cm in average.One case had a family history of nodular sclerosis.Three cases were found adipose tissue in CT scan and diagnosed for RAML,the other 3 cases were diagnosed for renal cancer.All cases were undergone surgical approach,3 cases were undergone resection of tumors only and the other 3 were performed nephrectomy. Results Pathological characters:tumor was mainly composed of epithelioid cells presented with invasive hyperplasia of atypical pleomorphism,hyperchromatic nuclei with frequent mitotic figures,giant neoplasic cells and extensive hemorrhagic necrosis immunohistochemcial staining showing a positive HMB45 and negative of EMA,CK in most neoplasic cells.All cases were followed up for 10 to 44 months,5 cases did not recurrence and metastases;the other had lung metastasis after operation 18 months later,without any treatment the patient died 10 months later. Conclusions Most of REAMLs are benign and often misdiagnosed for renal cancer by CT scan.HMB45 is positive in immunohistochemcial staining available for diagnose.The minority of REMAL is malignant potentially and should be followed up closely.Operation is major method.