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1、胰腺导管内乳头状黏液肿瘤(Intraductal papillary mucinous tumor),北大医院放射科 程晓悦,Patient,female,79-years old，withtumors in the body of the pancreas founded by the Ultrasound。CT shows that:Pancreaticatrophy；there were multipleround hypo-dense lesions in the neck and body of the pancreas，withclearboundaries and no enha
2、ncementin the enhanced CT scan；Some lesions had alittle strip separators and parts of thelesions wereclose to the main pancreatic duct；The pancreatic ductwas dilated.,定义,胰腺导管内乳头状黏液肿瘤(intraductal papillary mucinous tumor，IPMT)是一种特殊的胰腺囊腺瘤，可分泌大量黏液导致主胰管全程扩张，十二指肠乳头部开口由于黏液流过而扩大。相对少见的胰腺肿瘤。1982年由Ohashi首先报道，
3、此后陆续有一些报道，但对该病命名不同，如产黏液 癌、导管内癌、导管产黏液肿瘤等。1990年WHO将其统一称为IPMN（intraductal papillary mucinous neoplasms）。,特点,IPMT多见于60岁一70岁老年人，男性多于女性，而临床症状缺乏特异性，主要表现为反复上腹痛、乏力、纳差、消瘦及慢性胰腺炎、2型糖尿病等。特点：1、胰管内大量黏液潴留；2、乏特乳头部开口由于黏液流过而扩大；3、主要在主胰管发展和播散；4、很少有浸润的倾向；5、手术切除率高及预后良好等特点。,病理,IPMT的基本病理改变是胰管内分泌粘蛋白的上皮细胞乳头状增生，分泌大量黏液样物质并潴留于腺管
4、内造成胰管扩张。组织学上将其分为导管内乳头状黏液瘤、交界性和导管内乳头状黏液癌。根据肿瘤发生部位，通常把IPMT分为3型：主胰管型，肿瘤存在于主胰管并其扩张；分支胰管型，肿瘤位于分支胰管内；混合型，肿瘤既存在与主胰管又存在于分支胰管。,CT scan of the individual D:presence of a 20 mm BD-IPMN in the body of the pancreas(white arrow).,Main-duct intraductal papillary mucinous tumor(IPMT)with markedly dilated pancreatic
5、 duct with papillary projections that enhance on contrast-enhanced CT,MRCP：a cystic lesion in the uncinate process of the pancreas(asterisk)and a communicating branch duct(arrow)between the cystand the normal caliber main pancreatic duct.These findings are characteristic of a branch duct intraductal
6、 papillary mucinous neoplasm and this lesion has been stable on follow up MRCP examinations for 3 years.,ERCP shows opacification of the cystic lesion and the focally dilated main pancreatic duct near the cystic lesion.,影像表现,USCTMRIERCPMRCP。MRI在其分型方面优于CT。IPMT影像上主要表现为单房或多房囊性肿瘤，常伴有分隔及壁结节；增强扫描可见分隔及壁结节轻
7、-中度强化。分支管型好发于胰腺钩突，病变呈分叶状或葡萄状由多个直径12 cm的小囊聚合而成。少数也可融合为单一较大囊性改变，其内伴有索条状分隔。主胰管及分支胰管不同程度的扩张，在CT重建及MRCP中，可清晰显示病变与扩张腺管的关系，直接显示病变与扩张的胰管相通有利于本病的诊断与鉴别诊断。此外，IPMT常伴有胰腺的萎缩。,C,Helical CT scan shows communication(straight arrow)between dilated main pancreatic duct(curved arrow)and cystic lesion(arrowhead).D,Histo
8、logic specimen shows communication(straight arrow)between main pancreatic duct(curved arrow)and cystic lesion(arrowhead)covered by papillary epithelium smaller than 1 mm.(H and E,1),1.Natural history(1)Median age 6168 years(2)Patients with malignant IPMNs are about 5 years older as compared with tho
9、se with benign IPMNs2.Clinical symptoms(1)Obstructive jaundice(2)Epigastric pain(3)Weight loss(4)Diabetes3.Imaging1)The main duct and combined types of IPMNs have a higher risk of associated alignancy as compared with the branch-duct type2)Marked dilatation of the main pancreatic duct is associated
10、with malignancy in IPMNs3)Presence of thickening mural,large nodules or a solid mass is suggestive of malignancy in IPMNs4)IPMNs with common bile duct obstruction may indicate the occurrence of invasive cancer5)IPMNs invading adjacent structures,such as the duodenum,major vascular structures6)Lymph
11、node metastases,liver metastases or peritoneal deposits4.FNAC/B（细针穿刺活检）(1)Cytological examination of pancreatic juice(presence of malignant cells)identified as an independent predictor of invasive IPMNs(2)MUC1 expressionDiagnosis of malignant or invasive IPMNs.,CT检查对术前区分良、恶性IPMT,Chiu 等：CT片上发现主胰管明显扩张
12、、存在有附壁结节、厚的隔膜以及胰周界限不清等指标均为判定恶性IPMT的独立依据。Kawai 等：当肿瘤大小超过30 mm、附壁结节超过5 mm是诊断IPMT为恶性的一个重要依据。Sugiyama 等：恶性IPMT的主胰管直径扩张等于或大于7 mm可能提示为恶性。Kawamoto等：分析了46位IPMN患者的胰腺CT资料，发现主胰管扩张、主胰管受累、弥漫性或多发性病灶、壁内结节、肿瘤大小、胰管阻塞等都可作为判断肿瘤恶性行为的指标。,Axial T2-weighted(A)and subtraction(post-contrast minus precontrast)(B)images at th
13、e level of the pancreas：marked enlargementof the main pancreatic duct(arrowheads)with intraluminal enhancingpapillary projections(arrows).Main duct intraductal papillary mucinous neoplasm with in situ carcinoma was confirmed at histopathology after total pancreatectomy.Multiple renal cysts(asterisks
14、).,Fig.3.A 65-year-old woman with malignant IPMT with 12mm papillary neoplasms.a)CT：shows papillary neoplasms as slightly heterogeneous soft tissue in the dilated main pancreatic duct.b)Contrast-enhanced MR image:shows low signal intensity of the dilated main pancreatic duct andhypersignal intensity
15、 of papillary projections.c)MRCP:shows papillary neoplasmsas low signal intense areas in high signal intensity of the main pancreatic duct.,CT：the head of the pancreas shows a cystic lesion(asterisk)in the uncinate process of the pancreas with a hypo-attenuating area(arrow)in the adjacent pancreatic
16、 parenchyma.Note the intrahepatic biliary dilatation(arrowheads)due to obstruction of the common bile duct(not shown)by the infiltrating mass.Invasive pancreatic adenocarcinoma arising from an intraductal papillary mucinous neoplasm was confirmed at pathology after a Whipple procedure.GB:Gallbladder
17、.,Malignant IPMT of the pancreatic tail with invasion and occlusion of the splenic artery behind the pancreatic tail,a)Helical CT scan shows marked dilation of the main pancreatic duct and branch ducts.The dilated papilla bulges into the duodenal lumen(arrow).There is a ancreatoduodenalfistula(arrow
18、head).,b)Contrast-enhanced MR image(TR/TE,130/4.1)shows low signal intensity within the dilated main pancreatic duct and branch ducts.Bulging of the papilla into duodenal lumen(arrow)and a pancreatoduodenal fistula(arrowhead)are seen.,鉴别诊断,黏液性囊性肿瘤：是胰腺最常见的囊性肿瘤。多见于中老年女性大部分位于胰腺体尾部。一般肿瘤较大。圆形或卵圆形，单房或多房，内
19、见多少不一分隔及壁结节，囊壁可有钙化。周围有纤维包膜，内部以大囊性成分为主，主胰管一般不扩张。假囊肿多发生于急性胰腺炎后，位于胰周，囊壁相对较薄囊壁光滑，囊内无乳头状突起。如有以前影像学资料作为参考鉴别一般不难。浆液性囊腺瘤：成簇的多发小囊状结构，囊内液体密度更低，病变不与主胰管相通，直径多2 cm。典型者中心见星状纤维瘢痕样改变。,鉴别-黏液性囊腺瘤癌,Mucinous cystic neoplasm of the pancreatic body with a smooth round contour and septations,Mucinous cystadenocarcinoma in the pancreatic tail with mural projections and irregular septa,治疗,临床上对良、恶性IPMT 的治疗原则是明显不同的:对良性IPMT 肿瘤可采取最小限度的胰腺切除术(technique o f minimal pancreatectomy),对部分良性患者尤其是无明显症状者甚至可不必立即采取手术治疗而是密切观察;而对恶性IPMT 患者则必须立即手术治疗,必要时需扩大手术方式,其预后良好。,THANK YOU!,
