CAJ | 학술논문

目的探讨腹膜后血管平滑肌脂肪瘤(RAML)的临床病理学特征、诊断及鉴别诊断、治疗、预后等,以提高临床认识.方法回顾性分析2012-2014年收治的4例RAML患者的临床病理资料,运用全自动免疫组化仪进行AE1/AE3、Vimentin、HMB45、Melan-A、S-100、SMA、CD10、CD34、Ki-67免疫标记染色,同时进行随访调查,并复习相关文献.结果 4例RAML患者中女3例,男1例.3例有腹部疼痛不适的临床症状,1例体检时发现.肿物均大于10 cm,与肾脏界限不清.临床均行“肿物+患侧肾脏切除术”.光镜下肿瘤由脂肪、平滑肌细胞及厚壁血管构成,但三种成分比例相差较大,其中1例腹膜后肿物合并肾脏病变.免疫表型:AE 1/AE3均阴性;Vimentin、HMB45、Melan-A、SMA、CD34均阳性;S-100 3例阳性,1例阴性,Ki-67增殖指数均＜5％.结论 RAML是一种少见的腹膜后良性间叶性肿瘤,具有复杂多样的组织学形态,特殊的免疫表型结合镜下表现可明确其诊断.
목적 탐토복막후혈관평활기지방류(RAML)적림상병이학특정、진단급감별진단、치료、예후등,이제고림상인식.방법 회고성분석2012-2014년수치적4례RAML환자적림상병리자료,운용전자동면역조화의진행AE1/AE3、Vimentin、HMB45、Melan-A、S-100、SMA、CD10、CD34、Ki-67면역표기염색,동시진행수방조사,병복습상관문헌.결과 4례RAML환자중녀3례,남1례.3례유복부동통불괄적림상증상,1례체검시발현.종물균대우10 cm,여신장계한불청.림상균행“종물+환측신장절제술”.광경하종류유지방、평활기세포급후벽혈관구성,단삼충성분비례상차교대,기중1례복막후종물합병신장병변.면역표형:AE 1/AE3균음성;Vimentin、HMB45、Melan-A、SMA、CD34균양성;S-100 3례양성,1례음성,Ki-67증식지수균＜5％.결론 RAML시일충소견적복막후량성간협성종류,구유복잡다양적조직학형태,특수적면역표형결합경하표현가명학기진단.
Objective To analyze the clinical and pathological features,diagnosis and differential diagnosis,treatment and prognosis of retroperitoneal angiomyolipoma (RAML).Methods The clinical data of 3 cases diagnosed with RAML,during 2012 to 2014 were studied.The expression of AE1/AE3,Vimentin,HMB45,Melan-A,S-100,SMA,CD10,CD34 and Ki-67 were detected by full automatic immunohistochemistry instrument.Four cases were followed up,the relevant published articles were reviewed as well.Results Four patients contained 3 female and 1 male.Three patients of them were found because of abdominal pain and discomfort symptom,1 case was found on examination.All of them more than 10 cm,and the boundary was not clear."Tumor resection + nephrectomy" were used for treatment.Macroscopically,the tumor were consisted of fat,muscle cells and thick walled blood vessels.But they had different proportion.Immunohistochemically:AE1/AE3 negative; Vimentin,HMB45,Melan-A,SMA were positive; S-100 was positive in 3 cases and negative in 1 case; Ki-67 proliferation index were ＜5％.Conclusions The RAML is a rare benign retroperitoneal stromal tumor,which has a complex and diverse pathological organizations.The diagnosis can be made by special immune phenotype in combination with microscopic appearance.