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Cystic lesions of the pancreato-duodenal confluence. Who is who? Poster No.: C-0183 Congress: ECR 2014 Type: Educational Exhibit Authors: L. Goiburu Gonzalez , M. Paraira Beser , A. Pedrerol Perez , 1 3 1 2 2 2 J. A. de Marcos ; Terrassa, Barcelona/ES, Barcelona/ES, 3 TERRASSA/ES Keywords: Neoplasia, Inflammation, Cysts, Diagnostic procedure, Ultrasound, MR, CT, Pancreas, Gastrointestinal tract, Abdomen DOI: 10.1594/ecr2014/C-0183 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myESR.org Page 1 of 22 Learning objectives Our purposes are to illustrate the anatomy of the pancreato-duodenal region, and to describe the key radiological features of the different entities with a true cystic or a cysticlike component that can be found in this area. Background A wide spectrum of anomalies can be encountered at radiologic evaluation of the pancreato-duodenal junction. The hystology of the anatomic structures and pathology of different anomalies favor a true cystic or a cystic-like component at radiologic evaluation. From January 2009 through May 2012 we have retrospectively reviewed the radiological files of patients with cystic pancreatic lesions focusing on those located in the pancreatoduodenal confluence. We have revised the radiological signs on contrast enhanced-CT (CECT) and magnetic resonance imaging (MRI) with MR-cholangiopancreatography (MRCP) of each entity to characterize the lesions, to address the differential diagnosis and make a definite diagnosis whenever it has been possible. Normal anatomy Fig. 1 on page 3 Fig. 2 on page 3 - The pancreato-duodenal confluence is the region in which the duodenum, pancreatic head and distal common bile duct converge. It is located in the anterior pararenal space of the retroperitoneum. - In the pancreatic head we find the distal end of the common bile duct, the main pancreatic duct and accessory pancreatic ducts. - The major and minor papilla are located in the medial wall of the 2nd portion of the duodenum. - In the pancreaticoduodenal groove, we find the superior duodenopancreatic artery (gastroduodenal artery branch), which anastomoses with the inferior pancreaticoduodenal artery (branch of the superior mesenteric artery). The pancreaticoduodenal arteries establish the anatomical boundary between the head of the pancreas and duodenum. Page 2 of 22 We classified the findings as: 1.-Congenital and other entities: Duodenal diverticulum, choledochocele, santorinocele and duodenal duplication cyst. 2.-Inflammatory conditions: groove pancreatitis, focal pancreatitis and pancreatic pseudocyst. 3.-Tumoral pathology: Simple cyst, Cystoadenocarcinoma, Intraductal papillary mucinous neoplasm (IPMN), serous cystadenoma and mucinous cystic neoplasm. In all cases the final diagnosis was confirmed either pathologically or by stabilization of radiological findings for more than five years. Images for this section: Fig. 1: Axial CECT slices which show the superior mesenteric artery (red arrow) the gastroduodenal artery (yellow arrow) and the superior pancreatic duodenal artery (black arrow). Page 3 of 22 Fig. 2: Coronal CECT scans of the previous patient where we can observe the superior mesenteric artery (red arrow) the gastroduodenal artery (yellow arrow) and the superior pancreatic duodenal artery (black arrow). Page 4 of 22 Findings and procedure details Congenital and other entities: - Duodenal diverticulum Fig. 3 on page 11 • Most frequent location: periampullary region within a radius of 2 cm. • In 3% the major papilla is located inside • Communicates with the duodenal lumen • Radiological image: sacculation of the duodenal wall containing fluid and/or air. - Duodenal duplication cyst Fig. 4 on page 11 • It can be localized in any part of gastrointestinal tract. The medial wall of the second portion of the duodenum is a frequent site. • Generally it does not communicate with the duodenal lumen • Radiologic appearance: as a cystic lesion on ultrasound, CT and MRI. - Choledochocele • Type III biliary cyst Todani's classification. • Focal dilation of distal common bile duct. - Santorinocele • Cystic dilation of the distal dorsal pancreatic duct, proximal to the minor papilla. Inflammatory conditions: - Groove pancreatitis Page 5 of 22 • It is an uncommon form of focal chronic pancreatitis • Young males, alcohol abuse • Controversial pathogenesis: biliary disease, peptic ulcer, pancreatic heterotopia in the duodenal wall, anatomical or functional alteration in the minor papilla. • Two presentations: - Pure: affects only the groove - Segmental: affects also the pancreatic head PURE FORM image appearance Fig. 5 on page 11 CT MRI Flattened, hypodense lesion T1-WI hypointense lesion Generally does not produce dilation of the T2-WI varies depending on the evolution pancreatic duct Duodenal wall thickening with cystic lesions that are intramural or in the groove T2-WI hyperintense nodules in the duodenal wall Enhanced in delayed phases with iv contrast Enhanced in delayed phases with iv contrast SEGMENTAL FORM: Image appearance Fig. 6 on page 12 Fig. 7 on page 12 Fig. 8 on page 13 Fig. 9 on page 13 CT MRI/CPMRI Hypodense lesion in the pancreatic head Hypodense lesion in the pancreatic head on T1-WI Discrete dilated pancreatic and bile ducts Smooth and long segmental stenosis of the distal pancreatic duct and common bile duct Preservation of vascular structures Widening of the space between the distal pancreatic duct, common bile duct and duodenum lumen - Pseudocyst Fig. 10 on page 14 Fig. 11 on page 15 Page 6 of 22 • Most common pancreatic cystic lesion. • 4-6 weeks after onset of acute pancreatitis • Rounded, well-defined, hypodense lesion on CT. • The wall enhances after iv contrast administration WITHOUT intralesional enhancement. • MR imaging: hypointense on T1-WI, hyperintense on T2-WI, with areas of intermediate signal if it contains debris. • Look for pancreatic gland calcification. Tumoral pathology: - Ductal adenocarcinoma with cystic features Fig. 12 on page 15 Fig. 13 on page 16 Fig. 14 on page 17 • The cystic component has been described in 8% of ductal adenocarcinomas. • They can be associated with pseudocysts, dilated side branches or tumoral necrosis. -Intraductal papillary mucinous neoplasm (IPMN) Fig. 15 on page 18 Fig. 16 on page 18 Fig. 17 on page 19 • No sex predilection. 60-70 years old • Overproduction of mucin with progressive dilation of the ducts • Types: - Involving the main duct: • Diffuse or segmental dilation • 70% of malignancy - Involving secondary branches: • uni-or multifocal Page 7 of 22 • It can communicate with main pancreatic duct • Frequently in head / uncinate process • 20% of malignancy • NO calcified scar - Combined Form: • Dilation of the main duct and its branches. • Signs that suggest malignancy: - Involvement of the main duct - Diffuse (> 50%) or multifocal involvement of the main duct - Main duct dilation (> 6mm) - Solid component that enhances with iv contrast - Cyst size> 3.5cm - Intraluminal calcium - Serous cystic neoplasm • Benign neoplasm • 75% in women aged 60-70 years. • Formed by multiple microcysts (<1 cm), separated by thin septa forming a central scar that may be calcified. • Association with von Hippel-Lindau (multifocal) • It may displace / compress adjacent organs • It does not communicate with the main pancreatic duct • Variants: -Hypervascular or solid: (very rare) Page 8 of 22 • Very small cysts that can be demonstrated with MRI • Differential diagnosis: Neuroendocrine tumor -Macrocystic or oligocystic: (10%) • Differential Diagnosis: mucinous cystic neoplasm Radiologic image CT: hypodense lesion with thin septa, well-defined, multi-lobulated Fig. 18 on page 19 - Multiple small cysts (1-5mm of diameter) - Larger cysts (10-20 mm) in the periphery - 30% Calcified central scar - Central scar enhances in delayed post-contrast phase. - Highly vascularized septa with honeycomb appearance (20%) - No vascular involvement MRI: polilobulated nodule, formed by multiple microcysts, with high signal intensity on T2-WI Fig. 19 on page 19 - thin septa with low signal intensity - Hypointense central scar - Calcification produces a signal void in every sequence. - Post-Gd: Septa enhancement. - Delayed enhancement of the scar - Mucinous cystic neoplasm Fig. 20 on page 19 • Uncommon (2.5 %) among exocrine tumors • It has a high potential for malignancy (mucinous cystadenocarcinoma) • >95% females; 40-50 years of age. Page 9 of 22 • Mucin-producing cystic lesion immersed in ovarian-type stroma • Typically found in pancreatic body and tail • No communication with pancreatic duct. Radiologic image MRI: mutilocular(more frequently) or unilocular cystic lesion - Simple fluid signal: low signal on T1-WI and hyperintense on T2-WI - Cysts > 2cm separated by septa - Cyst walls and thick septa with delayed enhancement CT: well circumscribed hypodense lesion. - Multilocular (more frequently) or unilocular cystic lesion - Cysts > 2cm separated by septa - Walls and thick septa that enhances in with intravenous contrast. - Chronic pancreatitis changes distal to the tumor - 25% calcifications, typically peripheral •Signs that suggest malignancy - Enhancing mural nodules or papillary projections. - Calcifications - Cystic neuroendocrine tumor • Uncommon manifestation of a rare tumor. • Gastrinoma is the most common neuroendocrine tumor in this area, called the "gastrinoma triangle" • Caused by cystic degeneration. • Adults, 50 years, with no sex predilection Page 10 of 22 • Most frequently associated with MEN syndrome • Radiological image: well-defined nodule with a rim of highly vascularized tissue that shows avid enhancement in the early arterial phase. Images for this section: Fig. 3: Coronal and axial MRI T2 WI and CPMRI shows a structure with a fluid air level that appears to originate from the second portion of the duodenum consistent with duodenal diverticulum. Fig. 4: Axial and coronal MRI T2 WI. Cystic lesion in the medial wall of the descending duodenum. Page 11 of 22 Fig. 5: Groove pancreatitis pure form. Axial and coronal MRI T2-WI. Multiple cystic lesions in the duodenal wall with discrete duodenal narrowing. Pancreas is unaffected. The common bile duct and pancreatic duct are of normal caliber. Fig. 6: Acute segmental groove pancreatitis. Axial CECT images (A, B), coronal (D, E) and with oral contrast (C, F): There is a multilocular cystic lesion that affects the duodenum and pancreatic head. Note the adjacent fat stranding, without deforming or encompassing vascular structures. There is no biliary duct dilation. Minimal pancreatic duct dilation. Page 12 of 22 Fig. 7: Chronic phase of segmental groove pancreatitis. Axial CECT images in arterial phase: Thickening and hypodensity of the medial wall of the second portion of the duodenum is noted. Calcifications in the uncinate process of the pancreas. Intrahepatic biliary tract dilation. Vascular structures are unaffected. Retroaortic left renal vein. Fig. 8: Coronal CECT venous phase of the previous patient. Note the dilation of the common bile duct. The black arrow indicates the dilation of the Wirsung duct. The red arrow indicates a pseudocyst. Calcifications in the distal portion of the pancreatic duct. Hypodense nodules can be perceived in the thickened duodenal wall (yellow arrow). Page 13 of 22 Fig. 9: MR images in T2 WI (A, C, D and E) and T1 WI (B) of the previous patient, confirming cystic lesions in the duodenal wall. Page 14 of 22 Fig. 10: Pseudocyst. CECT images in axial and coronal sections. Rounded, hypodense, homogeneous, well-defined lesion in the pancreatic head with wall enhancement but without intralesional enhancement. It displaces vascular structures and compresses the duodenum and the bile duct. Multiple calcifications in the uncinate process indicating chronic pancreatitis Fig. 11: Coronal CECT image that shows another pseudocyst case with more severe chronic pancreatitis changes. Calcification in a dilated main pancreatic duct and marked pancreatic atrophy. Page 15 of 22 Fig. 12: 45 year old male A)Doppler ultrasound reveals an anechogenic lesion with internal echoes and focal thickening of the wall, located in the pancreatic head. B),C) and D)Ultrasound with intravenous contrast, clearly identifying a polypoid mass with early enhancement and early wash out of the contrast, consistent with a malignant process. Page 16 of 22 Fig. 13: MRI axial T2 WI (A, B) and coronal (C, D) of the same patient. We can observe a hyperintense mass (*) in the pancreato-duodenal junction region with focal wall thickening that causes dilation of the pancreatic and biliary ducts (double duct sign). Note in C the regular contour of the dilated pancreatic duct (black arrow). Page 17 of 22 Fig. 14: Axial CECT scans (A, B and C), coronal CECT scans (D, E) and sagittal MIP (F) of the prior patient, choledochal stent carrier, showing a hypodense mass (*) at the pancreato-duodenal confluence, which includes the common bile duct (yellow arrow) causing retrograde dilation of the bile duct (black arrow). There is not fat plane between the mass and the duodenum. The mass is encompassing the pancreato-duodenal artery (red arrow). Fig. 15: IPMN. Axial and coronal CECT scan images (A, B) and MR HASTE sequences (C, D, E). Observe the marked dilation of the pancreatic duct at uncinated process. There is also multifocal dilation of secondary branches in the pancreatic body and tail. Fig. 16: IPMN. CECT images in axial (A, B) and coronal (C) showing a hypodense lesion in the uncinate process, with a cystic appearance, well circumscribed, without calcifications or adjacent vessel involvement. No pancreatic duct dilatation. The arrow indicates the pancreaticoduodenal artery that helps us to locate the lesion in the pancreas. Page 18 of 22 Fig. 17: IPMN. Coronal MRI T2 weighted images of the prior patient shows that the uncinate process cystic-like lesion communicates with the pancreatic duct (arrow). The main pancreatic duct is not dilated. Minimum bile duct ectasia. Fig. 18: Serous cystic neoplasm. Axial (A) and coronal (B and C) CECT images. Mass in head/uncinate process, multicystic, well defined, with thin septa, which enhance after contrast administration (honeycomb appearance). Note displacement of adjacent structures. Cysts are small; the largest are seen in the periphery of the lesion. Fig. 19: Serous cystic neoplasm. MR images in T2-WI: Polylobulated nodule in the uncinate process formed by multiple hyperintense microcysts. Discrete dilation of the bile duct in a cholecystectomized patient. Main pancreatic duct not dilated. Page 19 of 22 Fig. 20: Mucinous cystic neoplasm. Axial and coronal CECT images which shows a lesion in the uncinate process. It is hypodense, well-defined, with septa, oligocystic and with peripheral calcifications. Page 20 of 22 Conclusion MRI and CECT are the imaging modalities of choice in the study of the pancreatoduodenal confluence. Knowledge of the normal anatomy and the typical radiological signs of the different entities involving this area is crucial to reach the proper diagnosis. Personal information References 1. Gourtsoyiannis, N., Clinical MRI of the Abdomen Why, How, When. Springer-Verlag Berlin Heidelberg 2011 (4) 225-280 2. Blasbalg., et al. MRI features of groove pancreatitis. AJR 2007 189 (1), 73-80. 3. Castell-Monsalve, F., et al. Groove pancreatitis: MRI and pathologic findings. Abdominal imaging, 2008 33(3), 342-8. 4. Triantopoulou, C., et al. Groove pancreatitis: a diagnostic challenge. European radiology, 2009 19(7), 1736-43 5. Govind, B. et al. 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