胰腺導(dǎo)管內(nèi)乳頭狀瘤.pptVIP

胰腺導(dǎo)管內(nèi)乳頭狀黏液腫瘤 (Intraductal papillary mucinous tumor),北大醫(yī)院放射科 程曉悅,Patient, female, 79-years old，with tumors in the body of the pancreas founded by the Ultrasound。 CT shows that: Pancreatic atrophy； there were multiple round hypo-dense lesions in the neck and body of the pancreas，with clear boundaries and no enhancement in the enhanced CT scan； Some lesions had a little strip separators and parts of the lesions were close to the main pancreatic duct； The pancreatic duct was dilated.,定義,胰腺導(dǎo)管內(nèi)乳頭狀黏液腫瘤(intraductal papillary mucinous tumor，IPMT)是一種特殊的胰腺囊腺瘤，可分泌大量黏液導(dǎo)致主胰管全程擴張，十二指腸乳頭部開口由于黏液流過而擴大。 相對少見的胰腺腫瘤 。1982年由Ohashi首先報道，此后陸續(xù)有一些報道，但對該病命名不同，如產(chǎn)黏液 癌、導(dǎo)管內(nèi)癌、導(dǎo)管產(chǎn)黏液腫瘤等。1990年WHO將其統(tǒng)一稱為IPMN（ intraductal papillary mucinous neoplasms ）。,特點,IPMT多見于60歲一70歲老年人，男性多于女性，而臨床癥狀缺乏特異性，主要表現(xiàn)為反復(fù)上腹痛、乏力、納差、消瘦及慢性胰腺炎、2型糖尿病等。 特點： 1、胰管內(nèi)大量黏液潴留； 2、乏特乳頭部開口由于黏液流過而擴大； 3、主要在主胰管發(fā)展和播散； 4、很少有浸潤的傾向； 5、手術(shù)切除率高及預(yù)后良好等特點。,病理,IPMT的基本病理改變是胰管內(nèi)分泌粘蛋白的上皮細胞乳頭狀增生，分泌大量黏液樣物質(zhì)并潴留于腺管內(nèi)造成胰管擴張。 組織學(xué)上將其分為導(dǎo)管內(nèi)乳頭狀黏液瘤、交界性和導(dǎo)管內(nèi)乳頭狀黏液癌。 根據(jù)腫瘤發(fā)生部位，通常把IPMT分為3型： 主胰管型，腫瘤存在于主胰管并其擴張； 分支胰管型，腫瘤位于分支胰管內(nèi)； 混合型，腫瘤既存在與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 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 cyst and the normal caliber main pancreatic duct. These findings are characteristic of a branch duct intraductal 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.,影像表現(xiàn),USCTMRIERCPMRCP。 MRI在其分型方面優(yōu)于CT。 IPMT影像上主要表現(xiàn)為單房或多房囊性腫瘤，常伴有分隔及壁結(jié)節(jié)；增強掃描可見分隔及壁結(jié)節(jié)輕-中度強化。 分支管型好發(fā)于胰腺鉤突，病變呈分葉狀或葡萄狀由多個直徑12 cm的小囊聚合而成。少數(shù)也可融合為單一較大囊性改變，其內(nèi)伴有索條狀分隔。 主胰管及分支胰管不同程度的擴張，在CT重建及MRCP中，可清晰顯示病變與擴張腺管的關(guān)系，直接顯示病變與擴張的胰管相通有利于本病的診斷與鑒別診斷。 此外，IPMT常伴有胰腺的萎縮。,C, Helical CT scan shows communication (straight arrow) between dilated main pancreatic duct (curved arrow) and cystic lesion (arrowhead). D, Histologic 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 those with benign IPMNs 2. Clinical symptoms (1) Obstructive jaundice(2) Epigastric pain(3) Weight loss(4) Diabetes 3. Imaging 1) The main duct and combined types of IPMNs have a higher risk of associated alignancy as compared with the branch-duct type 2) Marked dilatation of the main pancreatic duct is associated with malignancy in IPMNs 3) Presence of thickening mural, large nodules or a solid mass is suggestive of malignancy in IPMNs 4) IPMNs with common bile duct obstruction may indicate the occurrence of invasive cancer 5) IPMNs invading adjacent structures, such as the duodenum, major vascular structures 6) Lymph node metastases, liver metastases or peritoneal deposits 4. FNAC/B（細針穿刺活檢） (1) Cytological examination of pancreatic juice (presence of malignant cells) identified as an independent predictor of invasive IPMNs (2) MUC1 expression Diagnosis of malignant or invasive IPMNs.,CT檢查對術(shù)前區(qū)分良、惡性IPMT,Chiu 等：CT片上發(fā)現(xiàn)主胰管明顯擴張、存在有附壁結(jié)節(jié)、厚的隔膜以及胰周界限不清等指標(biāo)均為判定惡性IPMT的獨立依據(jù)。 Kawai 等：當(dāng)腫瘤大小超過30 mm、附壁結(jié)節(jié)超過5 mm是診斷IPMT為惡性的一個重要依據(jù)。 Sugiyama 等：惡性IPMT的主胰管直徑擴張等于或大于7 mm可能提示為惡性。 Kawamoto等：分析了46位IPMN患者的胰腺CT資料，發(fā)現(xiàn)主胰管擴張、主胰管受累、彌漫性或多發(fā)性病灶、壁內(nèi)結(jié)節(jié)、腫瘤大小、胰管阻塞等都可作為判斷腫瘤惡性行為的指標(biāo)。,Axial T2-weighted (A) and subtraction (post-contrast minus precontrast) (B) images at the level of the pancreas ： marked enlargement of the main pancreatic duct (arrowheads) with intraluminal enhancing papillary projections (arrows). Main duct intraductal papillary mucinous neoplasm with in situ carcinoma was confirmed at histopathology after total pancreatectomy. Multiple renal cysts (asterisks).,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 and hypersignal intensity of papillary projections. c)MRCP: shows papillary neoplasms as 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 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.,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 ancreatoduodenal fistula (arrowhead).,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.,鑒別