首页> 中文期刊> 《疑难病杂志》 >肝炎性肌纤维母细胞瘤949例临床分析

肝炎性肌纤维母细胞瘤949例临床分析

         

摘要

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.%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。

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