CAJ | 학술논문

目的分析肝血管平滑肌脂肪瘤(HAML)的临床病理特点、诊疗方法及预后.方法回顾性分析我院手术治疗的26例肝血管平滑肌脂肪瘤患者的临床病理资料.结果 26例患者中女性18例,男性8例,中位年龄43岁(27 ～ 66岁).14例(53.8％)无明显症状.本病术前影像学诊断困难,MRI及CT诊断准确率分别为57.1％和29.4％,高于超声诊断的16.7％(MRI:超声,x2=8.342,P=0.004;CT:超声,x2=4.329,P=0.037).所有患者均手术治疗,切缘为阴性.免疫组化检查:HMB45阳性率为100％ (21/21),SMA阳性率为100％(13/13),S-100阳性率为93.3％ (14/15).术后随访21～142个月(中位66个月),无复发和转移.结论 HAML好发于女性,缺乏典型的临床及影像表现;明确诊断依靠病理检查,HMB45是诊断HAML的重要标志,SMA和S-100也有助于HAML的诊断;手术切除是首选治疗方法,预后良好.
목적 분석간혈관평활기지방류(HAML)적림상병리특점、진료방법급예후.방법 회고성분석아원수술치료적26례간혈관평활기지방류환자적림상병리자료.결과 26례환자중녀성18례,남성8례,중위년령43세(27 ～ 66세).14례(53.8％)무명현증상.본병술전영상학진단곤난,MRI급CT진단준학솔분별위57.1％화29.4％,고우초성진단적16.7％(MRI:초성,x2=8.342,P=0.004;CT:초성,x2=4.329,P=0.037).소유환자균수술치료,절연위음성.면역조화검사:HMB45양성솔위100％ (21/21),SMA양성솔위100％(13/13),S-100양성솔위93.3％ (14/15).술후수방21～142개월(중위66개월),무복발화전이.결론 HAML호발우녀성,결핍전형적림상급영상표현;명학진단의고병리검사,HMB45시진단HAML적중요표지,SMA화S-100야유조우HAML적진단;수술절제시수선치료방법,예후량호.
Objective To study the clinical and pathological features of hepatic angiomyolipoma (HAML).Methods A retrospective study was conducted on 26 patients with pathologically-confirmed HAML who were treated in our hospital from January 2002 to December 2012.Results There were 8 males and 18 females,with a median age of 43 years (range 27-66 yr).Fourteen of 26 patients were asymptomatic at clinical presentation.MRI and CT had more conclusive findings (57.1％ and 29.4％ respectively) than ultrasound (US,16.7％),(MRI vs US:x2 =8.342,P=0.004; CT vs US:x2 =4.329,P=0.037).Surgical treatment with R0 resection was performed in all the patients.Immunohistochemistry analyses revealed positive stainings for HMB45,SMA and S-100 to be 100％ (21/21),100％ (13/13) and 93.3％ (14/15),respectively.During a follow-up of 21-142 months (median:66 months),no patient developed metastasis or recurrence.Conclusions HAML commonly occurred in women without any specific clinical manifestation.The diagnosis of HAML remained difficult and relied mainly on pathological findings.Although HMB-45 is considered as the main histopathological biomarker of HAML,SMA and S-100 may also be helpful in the diagnosis.Surgical resection is still the most optimal treatment,resulting in favorable prognosis for HAML.