肝脏少见恶性肿瘤影像课件.ppt

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,文档仅供参考，不能作为科学依据，请勿模仿；如有不当之处，请联系网站或本人删除。,肝脏恶性肿瘤,上皮性肿瘤,肝癌,肝内胆管细胞癌,胆管囊腺癌,肝癌与胆管癌,未分化癌,非上皮性肿瘤,上皮样血管内皮瘤,血管肉瘤,胚胎性肉瘤,横纹肌肉瘤,其他,混合性肿瘤,孤立性纤维瘤,畸胎瘤,卵黄囊肿瘤,癌肉瘤,Kaposi,肉瘤,横纹肌肿瘤,其他,造血和淋巴肿瘤,非何杰金氏淋巴瘤,继发性肿瘤,癌,淋巴瘤,肉瘤,PS,：最常见的肝脏恶性肿瘤为转移瘤；最常见的肝脏原发恶性肿瘤为,HCC,肝脏少见恶性肿瘤,肝脏胚胎性瘤（肝母细胞瘤）：,Hepatoblastoma,肝上皮样血管内皮细胞瘤,肉瘤,1%,血管肉瘤,胚胎性肉瘤,纤维肉瘤,平滑肌肉瘤,恶性纤维组织肉瘤,卵黄囊肉瘤,淋巴瘤,原发：,NHL,继发：,20%HL,，,50%NHL,肝母细胞瘤,儿童最常见的肝脏恶性肿瘤,占全部儿童恶性肿瘤的,1%,占小儿肝脏恶性肿瘤的,90%,发病年龄,0.08,岁,-8.74,岁,(,中位年龄,1,岁,),好发于,5,岁以下,90%,发生于,3,岁以前,成年人极为罕见,10%,肝母细胞瘤发生于早产儿,具有多种分化方式的恶性胚胎性肿瘤。它是由类似于胎儿性上皮性肝细胞、胚胎性细胞以及分化的间叶成分组成。,AFP,阳性,最多见于右叶,其次为左右叶,肝左叶最少见,通常形成单一肿块,少数为多结节,罕见弥漫侵及整个肝脏,肿瘤总钙化率为,38%50%,，大部分钙化聚集一处,此征象对肝母细胞瘤的诊断具有一定意义,肿瘤与正常肝分界清楚，肿瘤以外肝实质完全正常,在,T2WI,上可显示完整包膜,呈环状低信号影,肝母细胞瘤生长迅速,常于肿瘤边缘或中心部存在各种形态的出血、坏死、纤维瘢痕,瘤周尚可见绕行的肝血管影,主要为肝静脉和门静脉,多受压移位、管腔狭窄至闭塞,相邻器官受压变形。,影像特点,影像特点,-CT,影像特点,-MR,T2-weighted images demonstrated a 23 14 13 cm heterogeneous mass in the right lobe(a-b).,THRIVE images showed heterogeneous contrast enhanced with enhancement of fibrosis bands in tardive-phase(ce).,This heterogeneous mass occupied almost the whole right lobe of the liver presenting a well-defined capsule(f).,肝脏未分化肉瘤,亦称未分化或胚胎性肉瘤（,Undifferentiated enbryonal sarcoma,UES,），为发生于肝原始间叶组织的恶性肿瘤，主要发生在,6-10,岁的儿童，,90%,小于,15,岁，也可见于成人及新生儿,本病约占儿科肝脏恶性肿瘤的,13%,，占肝脏原发恶性肿瘤的,0.12%,临床特征通常是非特异性的，可表现为锐性腹痛、发热、厌食、腹泻或孤立性肝囊肿。通常没有黄疸。与原发性肝癌相比，,ULS,与肝炎或肝硬化无关。肝功能往往无异常，并且甲胎蛋白正常。,单发巨大肿块，囊性或囊实性，囊性成分多,实性成分位于边缘，结节状,实性成分表现为快进快出或延迟强化,出血常见,影像特点,肝上皮样血管内皮细胞瘤,少见的肝血管肿瘤，肿瘤生长缓慢，预后不一，生物学上为临界肿瘤，介于良性血管瘤和恶性血管瘤之间,好发于软组织，亦可发生于肺、肝、脾、骨及脑或全身多个部位同时发病，由,Weiss,等于,1982,年首次报道。,1984,年,Ishak,等首先报道了肝脏的,EHE,。,肝,EHE,病因不明，好发于中年女性，有学者认为与口服避孕药及接受雌激素治疗有关。,肝,EHE,临床表现无特异性，多为偶然发现，常见症状为右上腹部不适或疼痛，体重减轻，偶见黄疸，有时可有门静脉高压或,Budd-Chiari,综合征。,AFP,水平多在正常范围内。,肝,EHE,为低度恶性肿瘤，其预后明显好于血管肉瘤和肝细胞癌。转移少见，且多转移至肺部。文献有肝与肺同时发生,EHE,的报道，此时很难区分肺或肝内病灶是原发灶还是转移灶。,肝,EHE,多为肝内多发结节性病变，大多无手术机会，且对放疗及化疗不敏感，肝移植可能是肝,EHE,唯一的治愈手段,肝脏,EHE,病理上多表现为肝内多发结节性病灶，结节多位于肝包膜下，并可相互融合。结节切面呈灰白色，质地硬，无包膜，呈浸润性生长，周围质地正常，亦可充血。,镜下肿瘤由上皮样细胞及树突状细胞组成，两种细胞之间可见中间型细胞围绕血管腔周围。上皮样细胞沿肝窦呈浸润性生长，可向门静脉或肝静脉分支的腔内生长，瘤栓机化后可使血管腔闭塞。瘤体周围为富细胞区，中央为纤维区，纤维区可逐渐扩大，出现变性、出血、钙化。,主要表现为肝内多发结节，结节多位于肝周或肝包膜下，可以融合形成较大结节，邻近包膜可形成“包膜回缩征”,文献报道约,20%,的病灶内可见钙化,MR T1WI,呈低信号，,T2WI,呈中高混合信号，增强扫描病灶多为渐进性向中心延迟强化，动脉期病灶边缘轻度强化或无强化，门静脉期病灶逐渐向中心强化，但仍低于周围正常肝实质，文献中亦可呈等密度的报道,影像特点,影像特点,影像特点,肝血管肉瘤,原发性肝血管肉瘤（，）是最常见的肝脏恶性间质肿瘤，约占原发性肝脏肿瘤的，约占原发肝肿瘤的,0.4%,，在肝脏肉瘤中最多见，占,36%,。,其组织来源为血管或淋巴管上皮细胞。,预后极差，大多数患者平均生存期仅为个月。,临床症状和实验室检查无特异性。,根据的生长方式将其分为种,多发结节型、单发巨块型、巨块及结节混合型、弥漫浸润性微结节型。,影像特点,影像特点,恶性纤维组织肉瘤,Malignant fibrous histiocytosarcoma(MFH)is the mostcommon soft tissue sarcoma in adults,usually involvingthe deep fascia,extremities,or retroperitoneum,Originating in the liver is very unusual.It has five his-tological subtypes:storiform pleomorphic,giant cells,myxoid,inflammatory,and angiomatoid,HepaticMFH usually occurs in men between 50 and 60 years ofage.The 2-year survival rate is approximately 60%,and20%suffers from local recurrence,the mean survival time was 15 months.,CT findings of hepatic MFH include:,large,hetero-geneously enhancing mass with necrotic areas,Single enhancing peripheral pseudocapsule mass or cysticmass with cystic wall and fibrous septa enhancement,Better enhancement of the solid component and fibrous septa may be seen on delayed CT scan,MRI findings show the mass is with low-signal on T1WI and high-signal on T2WI.Enhanced MRI findings were the samewith CT scan,影像特点,影像特点,淋巴瘤,原发于肝脏的淋巴瘤，极为罕见，至目前为止仅见报道,30,余例。,Hepatic primary lymphoma constitutes about only,0.016%of nonHodgkins lymphoma(NHL)20.The,patients commonly present with right upper abdominal,quadrant discomfort or fever.,目前尚无严格的推荐治疗指南或共识，治疗方法主要包括外科手术、化疗、放疗及联合治疗。,对于病灶体积小、局限且肝功能尚可的病例，大多采用单独手术切除，预后较好，手术治疗可用于化疗前后以减轻肿瘤负荷，有报道非胃型,MALT,淋巴瘤,(,包括肝脏及其他部位,),术后复发率,(48,0%),明显高于胃,MALT,淋巴瘤,There are three morphologic patterns in hepatic lymphoma:,large solitary masses(4 cm),multiple focal nodules,diffuse infiltrative type.,Hepatic lymphoma appears as low-density lesions on unenhanced and contrast-enhanced CT scans,or has a thin enhancing rim.,The CT characteristics of hepatic secondary lymphoma include blood vessel floating sign and enhancement,影像特点,MRI findings show that the lesions present hypointense on T1WI and hyperintense on T2WI.,MRI can distinguish diffuse infiltration from normal liver tissue in cases in which neither sonography nor CT demonstrates any abnormalities,MRI can be used in NHL patients with a clinical suspicion of hepatic involvement when sonography and CT do not show any focal lesions.,影像特点,Positron emission tomography(PET)using deoxyglucose(FDG)scan has been performed for further evaluation,FDGPET/CT showed diffuse intense FDG uptake in the enlarged liver and spleen,with systemic FDG-avid lymphadenopathy including the hepatic hilar nodes,However,none of the imaging findings is specific for hepatic lymphoma.,影像特点,影像特点,谢谢,