腹膜后肾外及盆腔上皮样血管平滑肌脂肪瘤14例  

Retroperitoneal extrarenal or pelvic epithelioid angiomyolipoma:report of 14 cases and a literature review

作　　者：李文杰 苗成利[1] 张笑盈 高海成 邹博远 刘世博 罗成华 肖萌萌 Li Wenjie;Miao Chengli;Zhang Xiaoying;Gao Haicheng;Zou Boyuan;Liu Shibo;Luo Chenghua;Xiao Mengmeng(Department of Retroperitoneal Tumor Surgery,Peking University International Hospital,Beijing 102206,China;Department of Pathology,Peking University International Hospital,Beijing 102206,China;Department of General Surgery,Peking University People's Hospital,Beijing 100044,China)

机构地区：[1]北京大学国际医院腹膜后肿瘤外科,北京102206 [2]北京大学国际医院病理科,北京102206 [3]北京大学人民医院普通外科,北京100044

出　　处：《中华临床医师杂志(电子版)》2024年第10期888-894,共7页Chinese Journal of Clinicians(Electronic Edition)

基　　金：中国科协青年人才托举工程(2023QNRC001)

摘　　要：目的腹膜后肾外及盆腔上皮样血管平滑肌脂肪瘤在临床上较为少见,部分呈恶性生物样改变。由于缺乏特异性的征状和影像学表现,患者常延误诊治。本研究通过总结既往病例的诊疗经验并行文献复习,旨在提高对该疾病的认识。方法回顾性整理并分析北京大学国际医院2014年1月~2024年4月共14例行手术治疗且经病理确诊的腹膜后肾外及盆腔上皮样血管平滑肌脂肪瘤患者的临床资料,结合国内外文献对该疾病临床表现、影像学和病理学特征及诊疗进展进行总结。结果本组男性4例,女性10例。就诊时年龄20~67岁,中位年龄39岁。初发12例(体检发现4例,腹部不适5例,腰痛2例,排便障碍1例),复发2例。病灶位于腹膜后区域11例,盆腔3例。CT平扫呈稍低或等密度,瘤体较大时内部可有出血、坏死等表现;增强扫描实性成分呈不均匀明显强化。本组所有患者均行开腹手术治疗。术后病理提示恶性10例。免疫组化染色结果:HMB45阳性85.7%(12/14),MelanA阳性64.3%(9/14),SMA阳性91.7%(11/12)。术后并发症2例(腹腔出血1例,伤口感染1例),经保守治疗后好转。出院后规律随访,截至2024年7月,随访时间4~88个月,中位时间35个月。2例失访,1例(病例13)术后5月复查CT明确盆腔肿物复发(现口服西罗莫司治疗),1例(病例5)腹主动脉壁残留肿物未见明显增大,余患者均未见肿瘤复发及转移。结论腹膜后肾外及盆腔上皮样血管平滑肌脂肪瘤好发于中青年女性,临床表现不典型,术前影像学检查诊断准确率低,易与脂肪肉瘤、血管源性肿瘤等混淆。确诊依赖术后病理及免疫组化,特异性同时表达黑色素标志物(HMB45、Melan-A)及肌源性标志物(SMA)是其特征。治疗方案首选手术切除,但mTOR抑制剂的应用前景也较为客观。部分患者表现为恶性肿瘤学特征,可出现复发及远处转移,故术后需定期随访观察。Objective Retroperitoneal extrarenal or pelvic epithelioid angiomyolipomas are clinically rare,and some of them show malignant biological behavior.Due to the lack of specific symptoms and imaging findings,patients often experience a delayed diagnosis and treatment.This study aimed to improve the understanding of this disease by summarizing our diagnosis and treatment experience with 14 cases and reviewing the literature.Methods The clinical data of 14 patients with retroperitoneal extrarenal or pelvic epithelioid angiomyolipoma who underwent surgical treatment and achieved pathological confirmation in Peking University International Hospital from January 2014 to April 2024 were retrospectively analyzed.The clinical manifestations,imaging and pathological features,diagnosis,and treatment of the disease were summarized based on the domestic and foreign literature.Results There were 4 males and 10 females in this group.The patients ranged in age from 20 to 67 years at presentation,with a median age of 39 years.There were 12 cases of initial onset(4 cases discovered in physical examination,5 cases of abdominal discomfort,2 cases of low back pain,1 case of constipation)and 2 cases of recurrence.The lesions were located in retroperitoneal region in 11 cases and pelvic cavity in 3 cases.Plain CT scans showed slightly lower or equal density,and the tumor could have internal bleeding and necrosis when it was large.The solid component showed uneven and obvious enhancement.All patients in this group underwent open surgery.Postoperative pathology indicated malignancy in 10 cases.Immunohistochemical staining was positive for HMB45 in 85.7%(12/14)of patients,for MelanA in 64.3%(9/14),and for SMA in 91.7%(11/12).Postoperative complications in 2 cases(abdominal hemorrhage in 1 case and wound infection in 1 case)improved after conservative treatment.The patients underwent regular follow-up after discharge.As of July 2024,the follow-up time ranged from 4~88 months,with a median time of 35 months.Two cases were lost to follow-up.On

关 键 词：上皮样血管平滑肌脂肪瘤 诊断 手术 

分 类 号：R73[医药卫生—肿瘤] R737.3[医药卫生—临床医学]