罗含欢;霍真;王倩;尼玛;边巴扎西;
LUO Hanhuan;HUO Zhen;WANG Qian;NI Ma;BIANBA Zhaxi;Department of Pathology,Tibet Autonomous Region People's Hospital;Department of Pathology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;Department of Radiology,Tibet Autonomous Region People's Hospital;Department of General Surgery,Tibet Autonomous Region People's Hospital;

• 1:西藏自治区人民医院病理科
• 2:中国医学科学院北京协和医院病理科
• 3:西藏自治区人民医院放射科
• 4:西藏自治区人民医院普外科

摘要(Abstract)：

目的 对西藏地区泡状棘球蚴病(alveolar echinococcosis, AE)患者的临床表现和病理特征进行归纳、总结。方法 回顾性纳入2013年9月至2021年4月西藏自治区人民医院行手术治疗且病理资料完整的全部AE患者。通过电子病历系统提取其临床和病理资料，包括临床表现、影像学检查结果、病理结果、治疗方式以及预后等信息，并通过显微镜对病理切片进行重新阅片，总结其病理特征。结果 共纳入44例AE患者。其中男性17例，女性27例；平均年龄(36.2±12.3)岁；32例为农牧民。临床表现以消化系统症状最为常见(54.5%),其次为呼吸系统症状(13.6%),29.5%的患者无明显临床症状。CT检查示23例为单发病灶，21例为多发病灶(肝内多发病灶15例，多脏器受累6例);33例经CT检查诊断为AE。组织病理学示送检标本以巨块型为主，直径1.0～23.0 cm,切面呈蜂窝状。显微镜下可见病灶内存在大小、形状不一的小囊泡，其外层囊壁为薄层粉染角质层。囊泡以外生性方式增殖为主。早期病变可见坏死囊泡周围存在大量嗜酸性粒细胞浸润，伴肉芽肿形成；晚期陈旧性病变见粉尘状钙化和大片状坏死。44例均可见早期肉芽肿性病变和晚期陈旧性病变不同程度混合存在，其中5例伴脓肿形成。31例行根治性手术，13例行姑息性手术+药物治疗，随访2～82个月，死亡3例、存活41例。结论 西藏地区AE患者以青壮年农牧民为主，女性多见。肝脏为主要受累器官，少数病例可累及肺或多脏器。临床表现与受累脏器相关，以消化系统症状较常见。影像学检查对典型病例的辅助诊断具有较好的参考价值，非典型病例或合并脓肿时需通过病理检查确诊。病理检查可见泡状棘球蚴虫体呈球形囊泡状结构，且不同时期的病变常混合存在。经根治性手术治疗后多数患者预后良好。
Objective To summarize the clinical manifestations and pathological features of alveolarechinococcosis(AE) in Tibet, China. Methods Complete pathological data of all patients with surgical resection of AE in the Tibet Autonomous Region People's Hospital from September 2013 to April 2021 were retrospectively studied. Clinical and imaging findings, pathological results, treatment methods, and prognosis information were extracted through the electronic case system. The pathological sections were re-read by microscope to observe the pathological changes. Results A total of 44 AE patients were included. There were 17 males and 27 females; the average age was(36.2±12.3) years; 32 cases were farmers or herdsmen. The most common clinical manifestations were gastrointestinal symptoms(54.5%), followed by respiratory symptoms(13.6%), and 29.5% of patients had no obvious clinical symptoms. CT examination showed that 23 cases had a single lesion and 21 cases had multiple lesions(15 cases with multiple intrahepatic lesions and 6 cases with multiple organ involvement); 33 cases were diagnosed as AE by CT. The specimens for histopathological examination were mainly masses, with a diameter of 1.0-23.0 cm, and the sections were honeycombed. Microscopically, there were small vesicles of different sizes and shapes in the lesions, and the outer wall of the vesicles was a thin layer of pink stratum corneum. The proliferation of vesicles was mainly exogenous. In the early stage, a large number of eosinophils infiltrated around necrotic vesicles with the formation of granuloma; dust-like calcification and massive necrosis were seen in late old lesions. Early granulomatous lesions and late old lesions were mixed in all 44 cases, of which 5 cases were accompanied by abscess formation. A total of 31 cases underwent radical surgery, and 13 cases underwent palliative surgery and drug treatment. After a follow-up of 2-82 months, 3 cases died and 41 cases survived. Conclusions AE patients in China's Tibet are mainly young farmers and herdsmen, mostly women. The liver is the main organ involved, and a few cases can involve the lung or multiple organs. The clinical manifestations are related to the affected organs, and symptoms of the digestive system are more common. Imaging examination has a good reference value for the auxiliary diagnosis of typical cases, but atypical cases or cases complicated with abscess must be confirmed by pathology. Pathological examination shows that the body of alveolar echinococcus with a spherical vesicular structure, and the lesions of different periods often exist together. Most patients have a good prognosis by radical surgical treatment.

关键词(KeyWords)： 西藏地区;泡状棘球蚴病;临床表现;病理特征
Tibet region;alveolar echinococcosis;clinical manifestation;pathological feature

Abstract:

Keywords:

基金项目(Foundation):

作者(Authors): 罗含欢;霍真;王倩;尼玛;边巴扎西;
LUO Hanhuan;HUO Zhen;WANG Qian;NI Ma;BIANBA Zhaxi;Department of Pathology,Tibet Autonomous Region People's Hospital;Department of Pathology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;Department of Radiology,Tibet Autonomous Region People's Hospital;Department of General Surgery,Tibet Autonomous Region People's Hospital;

参考文献(References)：

• [1] 张耀刚，马艳艳，曹得萍，等.棘球属绦虫线粒体基因组全序列生物信息学分析[J].中国人兽共患病学报，2019,35:271- 277.
• [2] 陈哲宇.肝包虫病的治疗方法选择[J].中国临床医师杂志(电子版),2015,9:3329- 3331.
• [4] 四川省包虫病诊疗专家组.四川省肝包虫病诊治规范[J].中国普外基础与临床杂志，2017,24:798- 803.
• [5] 刘彤华。刘彤华诊断病理学[M].第4版.北京：人民卫生出版社，2018:300.
• [6] Qian MB,Chen J,Bergquist R,et al.Neglected tropical diseasesin the People's Republic of China:progress towards elimination[J].Infect Dis Poverty,2019,8:86.
• [7] 蒉嫣，伍卫平，韩帅，等.2018—2019年全国棘球蚴病监测分析[J].中国病原生物学杂志，2021,16:1025- 1029.
• [8] 伍卫平，王虎，王谦，等.2012—2016 年中国棘球蚴病抽样调查分析[J].中国寄生虫学与寄生虫病杂志，2018,36:1- 14.
• [9] 李海涛，吐尔干艾力，邵英梅，等.多器官泡状棘球蚴病的诊断治疗[J].中华消化外科杂志，2007,6:184- 187.
• [10] 谢亮，王姝，周林勇，等.川西藏区甘孜县肝棘球蚴病流行病学特征调查研究[J].中国普外基础与临床杂志，2020,27:38- 42.
• [11] Conraths FJ,Probst C,Possenti A,et al.Potential risk factors associated with human alveolar echinococcosis:systematic review and meta-analysis[J].PLoS Negl Trop Dis,2017,11:e0005801.
• [12] 方丹，陈哲宇.肝泡状棘球蚴病的诊断和治疗[J].临床肝胆病杂志，2017,33:990- 993.
• [13] 白翠，王楠，曹东阳，等.棘球蚴病概述[J].吉林畜牧兽医杂志，2017,2:53.
• [14] 赵兴华.对肝泡状棘球蚴病患者CT图像特征的分析[J].当代医药论丛，2017,15:133- 135.
• [15] 庄宇，余鑫，张菁，等.多层螺旋CT对肝泡状棘球蚴病的诊断与可切除性评价[J].中国普外基础与临床杂志，2016,23:104- 108.
• [16] 邢传平，刘斌，李宁，等.泡状棘球蚴病16例临床病理学及免疫组化观察[J].西北国防医学杂志，2005,26:353- 354.
• [17] Koch S,Bresson-Hadni S,Miguet JP,et al.Experienceof liver transplantation for incurable alveolar echinococcosis:a 45-case European collaborative report[J].Transplantation,2003,75:856- 863.
• [18] Kadry Z,Renner EC,Bachmann LM,et al.Evaluation oftreatment and long-term follow-up in patients with hepatic alveolarechinococcosis[J].Br J Surg,2005,92:1110- 1116.