疑难病杂志
疑難病雜誌
의난병잡지
JOURNAL OF DIFFICULT AND COMPLICATED CASES
2014年
6期
592-596
,共5页
李明信%陈育洪%陈晓东%钱民%廖秋林%马建青
李明信%陳育洪%陳曉東%錢民%廖鞦林%馬建青
리명신%진육홍%진효동%전민%료추림%마건청
炎性肌纤维母细胞瘤%肝脏%诊断%治疗
炎性肌纖維母細胞瘤%肝髒%診斷%治療
염성기섬유모세포류%간장%진단%치료
Inflammatory myofibroblastic tumor%Liver%Diagnosis%Treatment
目的：探讨肝炎性肌纤维母细胞瘤（ HIMT）的临床病理、诊断与治疗。方法收集949例HIMT（笔者诊治13例，文献报道936例）患者的临床资料，分析和总结其主要临床表现、影像学检查、病理学诊断依据及治疗方法与疗效。结果 HIMT常发生于成年男性，大部分以右上腹痛为首发，常伴有发热、乏力、消瘦等全身症状；实验室检查可有白细胞、甲胎蛋白（ AFP）升高；乙肝表现抗原阳性54例。影像学检查617例，其中547例（88 G．7％）单发局限性包块；B型超声检查409例，均为肝内不均匀回声包块，彩色多谱勒血流成像（ CDFI ）示：肿块内多无明显血供，少部分其内及周围探及血流信号，血流阻力指数（ RI）＜0．5；CT检查485例示：肝内均匀或不均匀低密度肿块，增强各期强化不明显，部分门脉及延迟期呈周边环状强化、中央核心样强化、分隔样强化等；MR检查216例示：T1W1序列多为低或稍低信号，T2W1序列多为高或稍高信号，动态增强扫描表现与CT相似。病理学检查：镜检可见增生的梭形细胞及浸润的慢性炎细胞包括淋巴细胞、浆细胞及胶原纤维形成；免疫组化示Vimentin、Actin、MSA、SMA、CD68等表达阳性555例（83．7％）患者行手术切除，手术方式有局部切除、肝段切除及半肝切除等。132例手术患者随访3个月～15年，1例术后1年复发，1例并发腹腔出血后治愈，1例合并肝硬化，并发肝衰竭死亡。99例（14．9％，99/663）行保守治疗，随访3个月～8年，部分病灶消失、缩小，部分无变化。结论 HIMT是较罕见的交界性间叶性肿瘤，临床无特征性，影像学检查有一定特征性，易误诊为恶性肿瘤，确诊需靠肝穿刺活检、手术病理学检查和免疫组化，局部切除和保守治疗是有效的治疗方法，预后良好，需长期随访。
目的：探討肝炎性肌纖維母細胞瘤（ HIMT）的臨床病理、診斷與治療。方法收集949例HIMT（筆者診治13例，文獻報道936例）患者的臨床資料，分析和總結其主要臨床錶現、影像學檢查、病理學診斷依據及治療方法與療效。結果 HIMT常髮生于成年男性，大部分以右上腹痛為首髮，常伴有髮熱、乏力、消瘦等全身癥狀；實驗室檢查可有白細胞、甲胎蛋白（ AFP）升高；乙肝錶現抗原暘性54例。影像學檢查617例，其中547例（88 G．7％）單髮跼限性包塊；B型超聲檢查409例，均為肝內不均勻迴聲包塊，綵色多譜勒血流成像（ CDFI ）示：腫塊內多無明顯血供，少部分其內及週圍探及血流信號，血流阻力指數（ RI）＜0．5；CT檢查485例示：肝內均勻或不均勻低密度腫塊，增彊各期彊化不明顯，部分門脈及延遲期呈週邊環狀彊化、中央覈心樣彊化、分隔樣彊化等；MR檢查216例示：T1W1序列多為低或稍低信號，T2W1序列多為高或稍高信號，動態增彊掃描錶現與CT相似。病理學檢查：鏡檢可見增生的梭形細胞及浸潤的慢性炎細胞包括淋巴細胞、漿細胞及膠原纖維形成；免疫組化示Vimentin、Actin、MSA、SMA、CD68等錶達暘性555例（83．7％）患者行手術切除，手術方式有跼部切除、肝段切除及半肝切除等。132例手術患者隨訪3箇月～15年，1例術後1年複髮，1例併髮腹腔齣血後治愈，1例閤併肝硬化，併髮肝衰竭死亡。99例（14．9％，99/663）行保守治療，隨訪3箇月～8年，部分病竈消失、縮小，部分無變化。結論 HIMT是較罕見的交界性間葉性腫瘤，臨床無特徵性，影像學檢查有一定特徵性，易誤診為噁性腫瘤，確診需靠肝穿刺活檢、手術病理學檢查和免疫組化，跼部切除和保守治療是有效的治療方法，預後良好，需長期隨訪。
목적：탐토간염성기섬유모세포류（ HIMT）적림상병리、진단여치료。방법수집949례HIMT（필자진치13례，문헌보도936례）환자적림상자료，분석화총결기주요림상표현、영상학검사、병이학진단의거급치료방법여료효。결과 HIMT상발생우성년남성，대부분이우상복통위수발，상반유발열、핍력、소수등전신증상；실험실검사가유백세포、갑태단백（ AFP）승고；을간표현항원양성54례。영상학검사617례，기중547례（88 G．7％）단발국한성포괴；B형초성검사409례，균위간내불균균회성포괴，채색다보륵혈류성상（ CDFI ）시：종괴내다무명현혈공，소부분기내급주위탐급혈류신호，혈류조력지수（ RI）＜0．5；CT검사485례시：간내균균혹불균균저밀도종괴，증강각기강화불명현，부분문맥급연지기정주변배상강화、중앙핵심양강화、분격양강화등；MR검사216례시：T1W1서렬다위저혹초저신호，T2W1서렬다위고혹초고신호，동태증강소묘표현여CT상사。병이학검사：경검가견증생적사형세포급침윤적만성염세포포괄림파세포、장세포급효원섬유형성；면역조화시Vimentin、Actin、MSA、SMA、CD68등표체양성555례（83．7％）환자행수술절제，수술방식유국부절제、간단절제급반간절제등。132례수술환자수방3개월～15년，1례술후1년복발，1례병발복강출혈후치유，1례합병간경화，병발간쇠갈사망。99례（14．9％，99/663）행보수치료，수방3개월～8년，부분병조소실、축소，부분무변화。결론 HIMT시교한견적교계성간협성종류，림상무특정성，영상학검사유일정특정성，역오진위악성종류，학진수고간천자활검、수술병이학검사화면역조화，국부절제화보수치료시유효적치료방법，예후량호，수장기수방。
Objective To investigate the inflammatory myofibroblastic tumor's ( HIMT) clinical pathology , diagnosis and treatment.Methods Collected 949 cases of HIMT (13 cases were diagnosed and treated by authors , 936 cases reported in the literature ) clinical data , analyze and summarize the main clinical manifestations , imaging , pathology , diagnosis and treatment method and effect .Results HIMT usually occurs in adult men , most of the right upper abdominal pain as the first , often accompanied by fever , fatigue, weight loss and other symptoms;laboratory examination can have white blood cells , in-creased AFP expression;hepatitis B antigen was positive in 54 cases.Imaging examination in 617 cases including 547 cases (88.7%) of solitary localized mass; B type ultrasound examination in 409 cases, intrahepatic inhomogeneous echo mass , CDFI showed no evident:within the tumor blood supply , a small part of it in and around the probe and signal of blood flow , blood flow resistance index ( RI) <0.5; CT examination of 485 cases of intrahepatic: homogeneous or inhomogeneous low density mass , the reinforcement is not obvious enhancement , part of the portal venous and delay phase showed a peripheral ring like enhancement , the central core like enhancement , separated like enhancement; MR examination 216 cases: T1W1 series low or slightly low signal , T2W1 sequence images revealed slightly higher signal , dynamic enhanced scan showed simi-lar to CT.Pathological examination: spindle cell microscopically visible hyperplasia and infiltration of chronic inflammatory cells including lymphocytes , plasma cells and collagen fibers formed;immunohistochemistry showed Vimentin , Actin, MSA, SMA, and CD68 expression was positive in 555 patients (83.7%) underwent surgical operation , operation mode local resec-tion, liver resection and half liver resection .132 cases of operation patients were followed up for 3 months to 15 years after op-eration, 1 cases recurred abdominal abdominal hemorrhage were cured , 1 cases died of cirrhosis, liver failure.In 99 cases (14.9%, 99/663) received conservative treatment,follow-up of 3 months to 8 years,some lesions disappeared,narrowed,some with no change.Conclusion HIMT is borderline rare mesenchymal tumor,without clinical features,imaging characteristics, easily misdiagnosed as malignant tumor , needs to be confirmed by liver biopsy , operation pathology and immunohistochemis-try, local resection or conservative treatment is , the prognosis is good , need long-term follow-up.
