DOI: 10.1148/rg.226025060
(Radiographics. 2002;22:1335-1352.)
© RSNA, 2002
Differential Diagnosis of Periampullary Carcinomas at MR Imaging1
Joo Hee Kim, MD,
Myeong-Jin Kim, MD,
Jae-Joon Chung, MD,
Woo Jung Lee, MD,
Hyung Sik Yoo, MD and
Jong Tae Lee, MD
1 From the Department of Diagnostic Radiology (J.H.K., M.J.K., J.J.C., H.S.Y., J.T.L.), the BK21 Project for Medical Science (M.J.K.), and the Department of Surgery (W.J.L.), Yonsei University College of Medicine, Seodaemun-ku, Shinchon-dong 134, Seoul 120-752, Korea. Recipient of a Certificate of Merit award for an education exhibit at the 2001 RSNA scientific assembly. Received March 18, 2002; revision requested April 4; final revision received August 27; accepted August 28. Address correspondence to M.J.K. (e-mail: kimnex@yumc.yonsei.ac.kr).
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Abstract
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Periampullary carcinomas arise within 2 cm of the major duodenal papilla and comprise carcinomas of the ampulla, distal common bile duct, pancreas, and duodenum. Their clinical features and anatomic locations are similar, as are the therapeutic approaches; however, their long-term outcomes vary. Magnetic resonance (MR) images of 89 pathologically proved periampullary carcinomas (29 ampullary carcinomas, 27 distal common bile duct carcinomas, 21 pancreatic carcinomas, six duodenal carcinomas, and six unclassified carcinomas) were reviewed. Ampullary carcinoma manifests as a small mass, periductal thickening, or bulging of the duodenal papilla. Pancreatic carcinoma is characterized by a discrete parenchymal mass, which enhances poorly on dynamic gadolinium-enhanced images. Sometimes, two proximal and two distal pancreatic and biliary ducts appear as four separate ducts (the four-segment sign). Dilatation of side branches of the pancreatic ducts is frequently seen in pancreatic carcinoma but not in other periampullary carcinomas. Distal bile duct carcinoma manifests as luminal obliteration and wall thickening or as an intraductal polypoid mass. A dilated proximal bile duct, a nondilated distal bile duct, and a dilated or nondilated pancreatic duct may form the three-segment sign. MR cholangiopancreatography and sectional MR imaging are useful in determining the origins of periampullary carcinomas.
© RSNA, 2002
Index Terms: Bile ducts, neoplasms, 766.321, 767.321 • Duodenum, neoplasms, 73.321 • Magnetic resonance (MR), cholangiopancreatography • Pancreas, neoplasms, 771.321
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LEARNING OBJECTIVES FOR TEST 2
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After reading this article and taking the test, the reader will be able to:
- Discuss the value of MR imaging in analysis of the origin and extent of periampullary carcinomas.
- Correlate the MR imaging features of periampullary carcinomas with the pathologic features.
- Describe the differential features of periampullary carcinomas on MR images.
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Introduction
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Periampullary carcinomas arise within 2 cm of the major papilla in the duodenum and include four different types of malignancies, namely, those originating from (a) the ampulla of Vater itself, (b) the intrapancreatic distal bile duct, (c) the head and uncinate process of the pancreas, and (d) the duodenum. Their origins are difficult and often impossible to discern based on clinical settings and results of preoperative imaging, as well as on surgical specimens (1–4).
Although periampullary tumors share similar clinical presentations, anatomic location, and therapeutic approaches (ie, classic or pylorus-preserving pancreaticoduodenectomy), their long-term outcomes may vary according to the specific type. Overall survival is highest for patients with ampullary and duodenal cancers, intermediate for patients with bile duct cancers, and lowest for those with pancreatic cancers (4–6).
We reviewed magnetic resonance (MR) cholangiopancreatographic (MRCP) images and sectional MR images of patients with periampullary carcinomas and defined the differential features of these tumors.
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Anatomy of the Ampulla of Vater
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Normally, the junction of the common bile duct (CBD) and the main pancreatic duct occurs in three ways: (a) a common duct (the ampulla of Vater) in 60% of cases, (b) a "double-barreled" opening at the apex of the papilla in 38% of cases, and (c) separate duodenal openings for the two canals in 2% of cases (7). Therefore, an ampulla of a common channel of the biliary and pancreatic ducts is not present in about 40% of cases. Occasionally, the two ducts unite and form a long common channel before entering the duodenal wall (8) (Fig 1).
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Figure 1a. Schematic drawings of the pancreaticobiliary junction. (a) A common duct with an ampulla. (b) A double-barreled opening at the apex of the papilla. (c) Separate duodenal openings for the biliary and pancreatic ducts. (d) A long common channel with pancreaticobiliary union outside the duodenal wall.
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Figure 1b. Schematic drawings of the pancreaticobiliary junction. (a) A common duct with an ampulla. (b) A double-barreled opening at the apex of the papilla. (c) Separate duodenal openings for the biliary and pancreatic ducts. (d) A long common channel with pancreaticobiliary union outside the duodenal wall.
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Figure 1c. Schematic drawings of the pancreaticobiliary junction. (a) A common duct with an ampulla. (b) A double-barreled opening at the apex of the papilla. (c) Separate duodenal openings for the biliary and pancreatic ducts. (d) A long common channel with pancreaticobiliary union outside the duodenal wall.
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Figure 1d. Schematic drawings of the pancreaticobiliary junction. (a) A common duct with an ampulla. (b) A double-barreled opening at the apex of the papilla. (c) Separate duodenal openings for the biliary and pancreatic ducts. (d) A long common channel with pancreaticobiliary union outside the duodenal wall.
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The ampulla of Vater comprises the junction of the biliary and pancreatic ducts and is surrounded by the sphincteric system of Oddi; it traverses a dehiscence of the duodenal wall and terminates at the major duodenal papilla (9). The sphincter of Oddi is a smooth muscle structure approximately 1 cm long that consists of the choledochal, pancreatic, and ampullary sphincters (10). The distal constricted end of the ampulla opens into the posteromedial aspect of the descending duodenum at the summit of the major duodenal papilla, covered by the duodenal mucosa (9,11). The major duodenal papilla is a polypoid prominence, 5–10 mm long and 5 mm wide, which is hidden by transverse, circular, and duodenal folds (7). In 75% of cases, the major duodenal papilla is in the descending duodenum. In these cases, the terminal pancreatic duct is inferior and anterior to the CBD (7). In 25% of cases, the major duodenal papilla is in the horizontal duodenum. In these cases, the pancreatic duct is positioned vertically and parallel to the left border of the CBD (7).
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Patients and MR Imaging Techniques
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Eighty-nine patients (61 men and 28 women; mean age, 59 years; age range, 37–78 years) with surgically proved periampullary carcinomas were included in our review. The carcinomas consisted of 29 ampullary carcinomas, 27 intrapancreatic distal CBD carcinomas, 21 pancreatic head or uncinate process carcinomas, six periampullary duodenal carcinomas, and six unclassified periampullary carcinomas. All patients underwent radical pancreaticoduodenectomy (Whipple operation) or pylorus-preserving pancreaticoduodenectomy.
All MR imaging examinations were performed with a 1.5-T system (Horizon; GE Medical Systems, Milwaukee, Wis) and a phased-array multi-coil. Detailed parameters for our MR imaging protocols are presented in the Table. In addition, serial consecutive single thick-slab MRCP images were obtained in selected patients (kinematic MRCP). Two radiologists jointly analyzed all MR images without knowledge of the final diagnosis. The appearances of ampullary, pancreatic, and biliary ductal masses and the shapes of the distal ends of the dilated ducts were analyzed. Dilatation of the CBD was defined as greater than 8 mm and dilatation of the main pancreatic duct was defined as greater than 4 mm on MRCP images.
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Ampullary Carcinoma
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Of the 29 patients with ampullary carcinoma, 18 (62%) had a discrete nodular mass, with an irregular filling defect, at the distal margin of the pancreaticobiliary junction (nodular type). Masses were 0.5–1.8 cm in diameter (mean, 1.0 cm) and were usually hypointense on T2-weighted images (Fig 2). In six patients (21%), irregular periductal thickening was seen around the pancreaticobiliary junction (periductal thickening type) (Fig 3). Papillary bulging into the duodenum was seen in 19 patients in both nodular (n = 14, 48%) and periductal thickening (n = 5, 17%) tumor types (Fig 4).
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Figure 2a. Ampullary carcinoma of the nodular mass type in a 49-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a nodular filling defect at the distal end of the CBD (arrow). Duodenal bulging of the ampulla is also seen. (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show a hypointense mass protruding into the duodenum (arrow).
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Figure 2b. Ampullary carcinoma of the nodular mass type in a 49-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a nodular filling defect at the distal end of the CBD (arrow). Duodenal bulging of the ampulla is also seen. (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show a hypointense mass protruding into the duodenum (arrow).
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Figure 2c. Ampullary carcinoma of the nodular mass type in a 49-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a nodular filling defect at the distal end of the CBD (arrow). Duodenal bulging of the ampulla is also seen. (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show a hypointense mass protruding into the duodenum (arrow).
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Figure 2d. Ampullary carcinoma of the nodular mass type in a 49-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a nodular filling defect at the distal end of the CBD (arrow). Duodenal bulging of the ampulla is also seen. (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show a hypointense mass protruding into the duodenum (arrow).
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Figure 3a. Ampullary carcinoma of the periductal thickening type in a 64-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows dilatation of the biliary tree and main pancreatic duct without a visible mass. (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show irregularity around the distal ends of the CBD (arrow) and pancreatic duct (arrowheads) with thickening of the ductal wall.
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Figure 3b. Ampullary carcinoma of the periductal thickening type in a 64-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows dilatation of the biliary tree and main pancreatic duct without a visible mass. (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show irregularity around the distal ends of the CBD (arrow) and pancreatic duct (arrowheads) with thickening of the ductal wall.
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Figure 3c. Ampullary carcinoma of the periductal thickening type in a 64-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows dilatation of the biliary tree and main pancreatic duct without a visible mass. (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show irregularity around the distal ends of the CBD (arrow) and pancreatic duct (arrowheads) with thickening of the ductal wall.
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Figure 3d. Ampullary carcinoma of the periductal thickening type in a 64-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows dilatation of the biliary tree and main pancreatic duct without a visible mass. (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show irregularity around the distal ends of the CBD (arrow) and pancreatic duct (arrowheads) with thickening of the ductal wall.
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Figure 4a. Ampullary carcinoma of the periductal thickening type with papillary bulging in a 60-year-old woman. (a) Schematic drawing. (b) Coronal T2-weighted SSFSE image shows irregular thickening of the ampullary margin (arrow). (c) Single-section MRCP image shows duodenal bulging of the ampulla (arrow). Convergence of the dilated bile and pancreatic ducts within 1 cm of the duodenal lumen is noted. (d) Coronal gadolinium-enhanced spoiled gradient-echo (GRE) image shows enlargement of the duodenal papillae and a poorly enhancing mass (arrow).
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Figure 4b. Ampullary carcinoma of the periductal thickening type with papillary bulging in a 60-year-old woman. (a) Schematic drawing. (b) Coronal T2-weighted SSFSE image shows irregular thickening of the ampullary margin (arrow). (c) Single-section MRCP image shows duodenal bulging of the ampulla (arrow). Convergence of the dilated bile and pancreatic ducts within 1 cm of the duodenal lumen is noted. (d) Coronal gadolinium-enhanced spoiled gradient-echo (GRE) image shows enlargement of the duodenal papillae and a poorly enhancing mass (arrow).
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Figure 4c. Ampullary carcinoma of the periductal thickening type with papillary bulging in a 60-year-old woman. (a) Schematic drawing. (b) Coronal T2-weighted SSFSE image shows irregular thickening of the ampullary margin (arrow). (c) Single-section MRCP image shows duodenal bulging of the ampulla (arrow). Convergence of the dilated bile and pancreatic ducts within 1 cm of the duodenal lumen is noted. (d) Coronal gadolinium-enhanced spoiled gradient-echo (GRE) image shows enlargement of the duodenal papillae and a poorly enhancing mass (arrow).
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Figure 4d. Ampullary carcinoma of the periductal thickening type with papillary bulging in a 60-year-old woman. (a) Schematic drawing. (b) Coronal T2-weighted SSFSE image shows irregular thickening of the ampullary margin (arrow). (c) Single-section MRCP image shows duodenal bulging of the ampulla (arrow). Convergence of the dilated bile and pancreatic ducts within 1 cm of the duodenal lumen is noted. (d) Coronal gadolinium-enhanced spoiled gradient-echo (GRE) image shows enlargement of the duodenal papillae and a poorly enhancing mass (arrow).
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Dilatation of both the biliary and pancreatic ducts (double-duct sign) was seen in 15 patients (52%) (Fig 3), while only the bile duct was dilated in the remaining 14 (Fig 2). The distance from the duodenal lumen to the ends of the dilated ducts was 2–9 mm (mean, 5 mm). In terms of the shape of the distal ends of the bile and pancreatic ducts, a blunted shape was most common, followed by a meniscus-like shape. The shape of the distal end of the dilated bile duct was found to frequently change in different projections or on serial MRCP images. Dilatation of the pancreatic side branches was rarely seen.
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Pancreatic Carcinoma
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Pancreatic masses were seen in 19 of 21 patients (90%) and were 1.0–2.5 cm in diameter (mean, 1.8 cm). In one patient, a pancreatic mass was initially regarded as a bile duct mass. In the other patient, no discernible mass was evident but bile duct wall thickening was seen. Pancreatic masses were usually hypointense or isointense on unenhanced T1-weighted images and poorly enhanced on dynamic gadolinium-enhanced images. On T2-weighted images, the masses were hypointense, isointense, or hyperintense, according to the degree of desmoplastic reaction or tumor necrosis.
The double-duct sign was seen in 13 patients (62%) (Fig 5). In seven of these patients (54%), the short segments of the biliary and pancreatic ducts distal to the obstruction were also seen. Therefore, the proximal biliary and pancreatic ducts and their distal parts appeared as four separate ducts. We called this appearance the four-segment sign. Seven pancreatic carcinomas (33%) showed dilatation of the bile duct without pancreatic ductal dilatation (Fig 6), and one showed pancreatic ductal dilatation without biliary dilatation (Fig 7). Dilatation of the side branches of the main pancreatic duct was frequently seen around the stenotic segment (n = 9, 43%). This finding was not observed in other periampullary carcinoma types. The distance from the duodenal lumen to the ends of the dilated ducts was 14–42 mm (mean, 25 mm). Various shapes of the distal margin of the pancreatic and bile ducts were seen, including blunted, beak, or rat-tail forms. The beak shape was the most common for the distal end of dilated bile ducts; the rat-tail shape, which was rarely seen in ampullary or bile duct carcinomas, was the most common for the distal end of dilated pancreatic ducts.
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Figure 5a. Pancreatic carcinoma with the double-duct sign and dilated side branches in a 52-year-old man. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal SSFSE (c) images show dilatation of the biliary tree and main pancreatic duct. The distal segments of the bile duct (large arrowhead) and pancreatic duct (arrow) are also seen, forming the four-segment sign. Dilated side branches of the pancreatic duct are seen around the stenotic segments (small arrowheads). (d) Axial gadolinium-enhanced spoiled GRE image shows a poorly enhancing mass (large arrow) around the CBD (small arrow) and pancreatic duct (arrowhead).
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Figure 5b. Pancreatic carcinoma with the double-duct sign and dilated side branches in a 52-year-old man. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal SSFSE (c) images show dilatation of the biliary tree and main pancreatic duct. The distal segments of the bile duct (large arrowhead) and pancreatic duct (arrow) are also seen, forming the four-segment sign. Dilated side branches of the pancreatic duct are seen around the stenotic segments (small arrowheads). (d) Axial gadolinium-enhanced spoiled GRE image shows a poorly enhancing mass (large arrow) around the CBD (small arrow) and pancreatic duct (arrowhead).
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Figure 5c. Pancreatic carcinoma with the double-duct sign and dilated side branches in a 52-year-old man. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal SSFSE (c) images show dilatation of the biliary tree and main pancreatic duct. The distal segments of the bile duct (large arrowhead) and pancreatic duct (arrow) are also seen, forming the four-segment sign. Dilated side branches of the pancreatic duct are seen around the stenotic segments (small arrowheads). (d) Axial gadolinium-enhanced spoiled GRE image shows a poorly enhancing mass (large arrow) around the CBD (small arrow) and pancreatic duct (arrowhead).
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Figure 5d. Pancreatic carcinoma with the double-duct sign and dilated side branches in a 52-year-old man. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal SSFSE (c) images show dilatation of the biliary tree and main pancreatic duct. The distal segments of the bile duct (large arrowhead) and pancreatic duct (arrow) are also seen, forming the four-segment sign. Dilated side branches of the pancreatic duct are seen around the stenotic segments (small arrowheads). (d) Axial gadolinium-enhanced spoiled GRE image shows a poorly enhancing mass (large arrow) around the CBD (small arrow) and pancreatic duct (arrowhead).
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Figure 6a. Pancreatic carcinoma with a dilated bile duct and a normal main pancreatic duct in a 60-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows marked bile duct dilatation with a normal main pancreatic duct. (c) Coronal gadolinium-enhanced spoiled GRE image shows a small extraluminal parenchymal mass adjacent to the distal bile duct (arrow).
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Figure 6b. Pancreatic carcinoma with a dilated bile duct and a normal main pancreatic duct in a 60-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows marked bile duct dilatation with a normal main pancreatic duct. (c) Coronal gadolinium-enhanced spoiled GRE image shows a small extraluminal parenchymal mass adjacent to the distal bile duct (arrow).
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Figure 6c. Pancreatic carcinoma with a dilated bile duct and a normal main pancreatic duct in a 60-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows marked bile duct dilatation with a normal main pancreatic duct. (c) Coronal gadolinium-enhanced spoiled GRE image shows a small extraluminal parenchymal mass adjacent to the distal bile duct (arrow).
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Figure 7a. Pancreatic carcinoma with a dilated pancreatic duct and a normal bile duct in a 65-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows short segmental narrowing of the distal main pancreatic duct (arrow). The extrahepatic bile duct is normal. (c, d) Coronal T2-weighted SSFSE (c) and axial gadolinium-enhanced spoiled GRE (d) images show a pancreatic parenchymal mass confined to the uncinate process (arrow).
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Figure 7b. Pancreatic carcinoma with a dilated pancreatic duct and a normal bile duct in a 65-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows short segmental narrowing of the distal main pancreatic duct (arrow). The extrahepatic bile duct is normal. (c, d) Coronal T2-weighted SSFSE (c) and axial gadolinium-enhanced spoiled GRE (d) images show a pancreatic parenchymal mass confined to the uncinate process (arrow).
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Figure 7c. Pancreatic carcinoma with a dilated pancreatic duct and a normal bile duct in a 65-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows short segmental narrowing of the distal main pancreatic duct (arrow). The extrahepatic bile duct is normal. (c, d) Coronal T2-weighted SSFSE (c) and axial gadolinium-enhanced spoiled GRE (d) images show a pancreatic parenchymal mass confined to the uncinate process (arrow).
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Figure 7d. Pancreatic carcinoma with a dilated pancreatic duct and a normal bile duct in a 65-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows short segmental narrowing of the distal main pancreatic duct (arrow). The extrahepatic bile duct is normal. (c, d) Coronal T2-weighted SSFSE (c) and axial gadolinium-enhanced spoiled GRE (d) images show a pancreatic parenchymal mass confined to the uncinate process (arrow).
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Distal CBD Carcinoma
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Of the 27 patients with distal bile duct cancer, 24 (89%) had ductal wall thickening with luminal obliteration (obstructive type) (Fig 8). In three cases (11%), an intraductal polypoid mass was present without complete obliteration of the lumen (polypoid type) (Fig 9).
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Figure 8a. Distal CBD carcinoma of the obstructive type in a 38-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a markedly dilated CBD. There is mild dilatation of the main pancreatic duct in the absence of obstruction (arrow). The distal segment of the bile duct below the obstructive lesion is also seen (arrowhead), forming the three-segment sign. (c) Coronal T2-weighted SSFSE image shows abrupt narrowing of the intrapancreatic segment of the CBD. (d) Axial T2-weighted SSFSE image shows the pancreatic duct (arrow), but the narrowed bile duct is not seen. (e) Axial T1-weighted spoiled GRE image shows the thickened wall of the bile duct (arrowhead). The pancreatic duct is also seen (arrow). (f) Axial gadolinium-enhanced image shows mild enhancement of the thickened bile duct wall (arrowhead), as well as the pancreatic duct (arrow).
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Figure 8b. Distal CBD carcinoma of the obstructive type in a 38-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a markedly dilated CBD. There is mild dilatation of the main pancreatic duct in the absence of obstruction (arrow). The distal segment of the bile duct below the obstructive lesion is also seen (arrowhead), forming the three-segment sign. (c) Coronal T2-weighted SSFSE image shows abrupt narrowing of the intrapancreatic segment of the CBD. (d) Axial T2-weighted SSFSE image shows the pancreatic duct (arrow), but the narrowed bile duct is not seen. (e) Axial T1-weighted spoiled GRE image shows the thickened wall of the bile duct (arrowhead). The pancreatic duct is also seen (arrow). (f) Axial gadolinium-enhanced image shows mild enhancement of the thickened bile duct wall (arrowhead), as well as the pancreatic duct (arrow).
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Figure 8c. Distal CBD carcinoma of the obstructive type in a 38-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a markedly dilated CBD. There is mild dilatation of the main pancreatic duct in the absence of obstruction (arrow). The distal segment of the bile duct below the obstructive lesion is also seen (arrowhead), forming the three-segment sign. (c) Coronal T2-weighted SSFSE image shows abrupt narrowing of the intrapancreatic segment of the CBD. (d) Axial T2-weighted SSFSE image shows the pancreatic duct (arrow), but the narrowed bile duct is not seen. (e) Axial T1-weighted spoiled GRE image shows the thickened wall of the bile duct (arrowhead). The pancreatic duct is also seen (arrow). (f) Axial gadolinium-enhanced image shows mild enhancement of the thickened bile duct wall (arrowhead), as well as the pancreatic duct (arrow).
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Figure 8d. Distal CBD carcinoma of the obstructive type in a 38-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a markedly dilated CBD. There is mild dilatation of the main pancreatic duct in the absence of obstruction (arrow). The distal segment of the bile duct below the obstructive lesion is also seen (arrowhead), forming the three-segment sign. (c) Coronal T2-weighted SSFSE image shows abrupt narrowing of the intrapancreatic segment of the CBD. (d) Axial T2-weighted SSFSE image shows the pancreatic duct (arrow), but the narrowed bile duct is not seen. (e) Axial T1-weighted spoiled GRE image shows the thickened wall of the bile duct (arrowhead). The pancreatic duct is also seen (arrow). (f) Axial gadolinium-enhanced image shows mild enhancement of the thickened bile duct wall (arrowhead), as well as the pancreatic duct (arrow).
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Figure 8e. Distal CBD carcinoma of the obstructive type in a 38-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a markedly dilated CBD. There is mild dilatation of the main pancreatic duct in the absence of obstruction (arrow). The distal segment of the bile duct below the obstructive lesion is also seen (arrowhead), forming the three-segment sign. (c) Coronal T2-weighted SSFSE image shows abrupt narrowing of the intrapancreatic segment of the CBD. (d) Axial T2-weighted SSFSE image shows the pancreatic duct (arrow), but the narrowed bile duct is not seen. (e) Axial T1-weighted spoiled GRE image shows the thickened wall of the bile duct (arrowhead). The pancreatic duct is also seen (arrow). (f) Axial gadolinium-enhanced image shows mild enhancement of the thickened bile duct wall (arrowhead), as well as the pancreatic duct (arrow).
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Figure 8f. Distal CBD carcinoma of the obstructive type in a 38-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a markedly dilated CBD. There is mild dilatation of the main pancreatic duct in the absence of obstruction (arrow). The distal segment of the bile duct below the obstructive lesion is also seen (arrowhead), forming the three-segment sign. (c) Coronal T2-weighted SSFSE image shows abrupt narrowing of the intrapancreatic segment of the CBD. (d) Axial T2-weighted SSFSE image shows the pancreatic duct (arrow), but the narrowed bile duct is not seen. (e) Axial T1-weighted spoiled GRE image shows the thickened wall of the bile duct (arrowhead). The pancreatic duct is also seen (arrow). (f) Axial gadolinium-enhanced image shows mild enhancement of the thickened bile duct wall (arrowhead), as well as the pancreatic duct (arrow).
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Figure 9a. Distal CBD carcinoma of the polypoid type in a 57-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a lobulated filling defect in the dilated distal CBD (arrow). (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show an intraluminal papillary mass in the distal bile duct (arrow).
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Figure 9b. Distal CBD carcinoma of the polypoid type in a 57-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a lobulated filling defect in the dilated distal CBD (arrow). (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show an intraluminal papillary mass in the distal bile duct (arrow).
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Figure 9c. Distal CBD carcinoma of the polypoid type in a 57-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a lobulated filling defect in the dilated distal CBD (arrow). (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show an intraluminal papillary mass in the distal bile duct (arrow).
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Figure 9d. Distal CBD carcinoma of the polypoid type in a 57-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows a lobulated filling defect in the dilated distal CBD (arrow). (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show an intraluminal papillary mass in the distal bile duct (arrow).
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CBD dilatation was found in 26 patients (96%). The pancreatic duct was dilated in three patients, in whom tumor extension to the pancreatic parenchyma or the ampulla was proved pathologically. Frequently (n = 15, 56%), the distal segment of the bile duct below the obstruction was also seen on MRCP images; hence, the biliary and pancreatic ducts were seen in three segments (three-segment sign) (Fig 8).
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Periampullary Duodenal Carcinoma
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In three of the six patients with duodenal carcinomas, both the biliary and pancreatic ducts were normal. However, in the remaining three patients, variable degrees of bile and pancreatic ductal dilatation were seen. Primary tumors were correctly identified on MR images in five patients: a small polypoid mass in three (Fig 10), a fungating mass in one (Fig 11), and a scirrhous carcinoma with eccentric duodenal wall thickening and luminal narrowing in one (Fig 12). In one patient with no discernible mass, kinematic MRCP revealed persistent contraction of the sphincteric segments, which suggested the presence of periampullary disease.
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Figure 10a. Periampullary duodenal carcinoma of the polypoid type in a 57-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows normal bile and pancreatic ducts. A polypoid filling defect is seen in the periampullary duodenal lumen (arrow). (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show a polypoid mass at the duodenal papilla protruding into the duodenal lumen (arrow).
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Figure 10b. Periampullary duodenal carcinoma of the polypoid type in a 57-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows normal bile and pancreatic ducts. A polypoid filling defect is seen in the periampullary duodenal lumen (arrow). (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show a polypoid mass at the duodenal papilla protruding into the duodenal lumen (arrow).
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Figure 10c. Periampullary duodenal carcinoma of the polypoid type in a 57-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows normal bile and pancreatic ducts. A polypoid filling defect is seen in the periampullary duodenal lumen (arrow). (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show a polypoid mass at the duodenal papilla protruding into the duodenal lumen (arrow).
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Figure 10d. Periampullary duodenal carcinoma of the polypoid type in a 57-year-old man. (a) Schematic drawing. (b) Single-section MRCP image shows normal bile and pancreatic ducts. A polypoid filling defect is seen in the periampullary duodenal lumen (arrow). (c, d) Coronal (c) and axial (d) T2-weighted SSFSE images show a polypoid mass at the duodenal papilla protruding into the duodenal lumen (arrow).
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Figure 11a. Periampullary duodenal carcinoma of the fungating type in a 56-year-old woman. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal T2-weighted SSFSE (c) images show a lobulated polypoid mass (arrow) at the duodenal papilla extending along the duodenal wall to the infrapapillary area. Mild dilatation of the bile duct is also seen. (d) Endoscopic image of the periampullary area shows the mass.
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Figure 11b. Periampullary duodenal carcinoma of the fungating type in a 56-year-old woman. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal T2-weighted SSFSE (c) images show a lobulated polypoid mass (arrow) at the duodenal papilla extending along the duodenal wall to the infrapapillary area. Mild dilatation of the bile duct is also seen. (d) Endoscopic image of the periampullary area shows the mass.
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Figure 11c. Periampullary duodenal carcinoma of the fungating type in a 56-year-old woman. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal T2-weighted SSFSE (c) images show a lobulated polypoid mass (arrow) at the duodenal papilla extending along the duodenal wall to the infrapapillary area. Mild dilatation of the bile duct is also seen. (d) Endoscopic image of the periampullary area shows the mass.
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Figure 11d. Periampullary duodenal carcinoma of the fungating type in a 56-year-old woman. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal T2-weighted SSFSE (c) images show a lobulated polypoid mass (arrow) at the duodenal papilla extending along the duodenal wall to the infrapapillary area. Mild dilatation of the bile duct is also seen. (d) Endoscopic image of the periampullary area shows the mass.
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Figure 12a. Periampullary duodenal carcinoma of the annular constrictive type in a 58-year-old man. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal T2-weighted SSFSE (c) images show irregular thickening and narrowing of the duodenum at the junction of the second and third portions (arrow). Owing to stasis, fluid-filled distention of the stomach and proximal duodenum is seen. (d) Coronal gadolinium-enhanced spoiled GRE image shows an enhancing mass infiltrating the pancreatic parenchyma near the ampulla (arrow).
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Figure 12b. Periampullary duodenal carcinoma of the annular constrictive type in a 58-year-old man. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal T2-weighted SSFSE (c) images show irregular thickening and narrowing of the duodenum at the junction of the second and third portions (arrow). Owing to stasis, fluid-filled distention of the stomach and proximal duodenum is seen. (d) Coronal gadolinium-enhanced spoiled GRE image shows an enhancing mass infiltrating the pancreatic parenchyma near the ampulla (arrow).
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Figure 12c. Periampullary duodenal carcinoma of the annular constrictive type in a 58-year-old man. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal T2-weighted SSFSE (c) images show irregular thickening and narrowing of the duodenum at the junction of the second and third portions (arrow). Owing to stasis, fluid-filled distention of the stomach and proximal duodenum is seen. (d) Coronal gadolinium-enhanced spoiled GRE image shows an enhancing mass infiltrating the pancreatic parenchyma near the ampulla (arrow).
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Figure 12d. Periampullary duodenal carcinoma of the annular constrictive type in a 58-year-old man. (a) Schematic drawing. (b, c) Single-section MRCP (b) and coronal T2-weighted SSFSE (c) images show irregular thickening and narrowing of the duodenum at the junction of the second and third portions (arrow). Owing to stasis, fluid-filled distention of the stomach and proximal duodenum is seen. (d) Coronal gadolinium-enhanced spoiled GRE image shows an enhancing mass infiltrating the pancreatic parenchyma near the ampulla (arrow).
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Discussion
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Recent studies have shown that there are inherent differences in the tumor biologies of the different types of periampullary carcinomas, which account for the observed survival differences (1,2,5). Accurate diagnosis and origin determination may influence treatment planning and the prediction of prognosis. In comparison to pancreatic cancer, distal bile duct cancer is more resectable and less frequently demonstrates tumor spread to adjacent lymph nodes or the resection margin microscopically (12,13). To improve survival, the radical resection of pancreatic cancer should be more frequently combined with chemoradiation therapy or more extensive regional lymphadenectomy than the radical resection of nonpancreatic periampullary cancers (14–17). Meanwhile, patients with ampullary or duodenal carcinoma, even with nodal metastasis, have better 5-year survival rates than those with bile duct or pancreatic carcinoma (18,19). For this reason, aggressive resection is indicated in ampullary or duodenal carcinoma, even in the presence of positive lymph nodes at preoperative imaging.
MRCP has evolved as an accurate diagnostic modality for the evaluation of pancreaticobiliary diseases; however, there is still a limitation in the evaluation of periampullary disease. This is because of the small but relatively complex anatomy of this region and because the tapered area of the distal biliary and pancreatic ducts contains little or no fluid. Physiologic contraction of the sphincter of Oddi also makes it difficult to evaluate the periampullary area. The combination of MRCP with conventional T1- and T2-weighted MR imaging, including gadolinium-enhanced dynamic MR imaging, is important for the evaluation of periampullary disease in terms of both detection and evaluation of the extent of a periampullary mass (20–22). Acquisition of serial single-section MRCP images (kinematic MRCP) may also provide useful information for differentiating between pathologic stenosis and physiologic narrowing of the sphincteric segment (23,24).
Ampullary carcinomas arise from the glandular epithelium of the ampulla of Vater (11). In some textbooks, this tumor is described as a bile duct cancer, arising in the last centimeter of the CBD (3). However, recent reports advocated that ampullary carcinoma should be classified as a duodenal cancer because ampullary and duodenal cancers share the same molecular development and the clinical outcomes of both are better than those of bile duct and pancreatic cancer (1,2). In addition, approximately 40% of the population does not have the common ampulla itself, and the biliary and pancreatic ducts drain into the duodenum separately.
Ampullary carcinomas typically manifest as small tumors at the time of diagnosis because of their relatively early symptom onset, and the mass itself is often inapparent at imaging (20,21). Jaundice develops when a tumor is small and precedes other common symptoms such as nausea, vomiting, or abdominal pain in patients with ampullary carcinoma. There are several reasons that may explain the better prognosis of ampullary carcinoma than other types of periampullary carcinomas. Ampullary carcinomas have a tendency toward intraluminal growth and frequently manifest with a polypoid or papillary form, and extraluminal extension is relatively infrequent. Papillary or polypoid cancers, which tend to have a better prognosis than invasive or infiltrative cancers, are more common in ampullary and duodenal carcinomas. An undifferentiated histologic type is less common in ampullary carcinoma than in the others. Lymphatic spread and perineural invasion are also infrequent in ampullary carcinoma. Investigators suggest that the genetic differences between periampullary carcinomas probably contribute to the phenotypic and clinical difference (1).
When a mass is depicted at cross-sectional imaging, it appears to protrude into the duodenal lumen. An irregularity of the inner margin of the distal biliary or pancreatic duct is useful for differentiating between choledocholithiasis and a benign obstruction. On MR images, the masses usually appear nearly isointense or hypointense on T1- and T2-weighted images and are poorly or moderately enhanced. Therefore, changes in signal intensity characteristics cannot reliably indicate malignancy, and differentiation of ampullary carcinomas from rare benign tumors is difficult with only imaging findings.
When a mass is not seen on MR images, a prominence or bulging appearance of the duodenal papilla, formed by protrusion(s) of the dilated biliary or pancreatic duct, may be the only sign of ampullary carcinoma. In this situation, benign biliary dilatation due to biliary dyskinesia, benign ampullary stenosis (either idiopathic or secondary to choledocholithiasis), or pancreatitis is difficult to differentiate. Sometimes, a bulging papilla, caused by a dilated bile duct, may also mimic a choledochocele (25). Therefore, secondary choledochocele from obstruction of the ampullary opening by a small ampullary tumor should be differentiated from other conditions of cystic dilatation of the distal CBD. Marked and abrupt dilatation of the distal bile duct or the pancreatic duct in the absence of stone disease or pancreatitis is suggestive of ampullary carcinoma.
Pancreatic carcinomas have the highest frequencies of larger size, poor histologic differentiation, and lymphatic and perineural extension among periampullary carcinomas (1–6). In cases of pancreatic carcinoma, the depiction of pancreatic parenchymal masses at MR imaging is useful for differentiating these tumors from other types of periampullary carcinomas. Pancreatic masses are usually more clearly delineated on gadolinium-enhanced spoiled GRE images than on unenhanced T1- or T2-weighted images (20).
There are some differential indicators of the classic double-duct sign (dilatation of both the pancreatic and bile ducts) for ampullary carcinomas and pancreatic carcinomas. In pancreatic carcinoma, we found that the most distal parts of the pancreatic and bile ducts are relatively spared from tumor infiltration; hence, four segments (two bile duct segments and two pancreatic duct segments) are visualized. We called this the four-segment sign. This sign was rarely seen in other types of periampullary carcinomas. A similar finding may rarely be seen in cases of chronic pancreatitis; however, the narrowing of the pancreatic duct is incomplete and relatively longer in cases of chronic pancreatitis in our experience. Another characteristic finding of pancreatic carcinoma at MRCP was dilatation of small branches of the pancreatic duct. The distance from the duodenal lumen to the stenotic segment was also relatively longer in pancreatic carcinomas than in ampullary carcinomas.
In the present study, bile duct carcinomas manifested as ductal wall thickening with luminal obliteration or as intraluminal polypoid masses. The involved segment usually appeared as fibrotic thickening of low signal intensity with narrowing and showed delayed enhancement on gadolinium-enhanced MR images. MRCP revealed abrupt tapering of the distal CBD. In cases of nodular or infiltrative cholangiocarcinomas, a narrowed lumen was well depicted on MRCP or T2-weighted SSFSE images, but the thickened ductal wall was better visualized on gadolinium-enhanced dynamic MR images. In cases of the polypoid type, the masses were well visualized on MRCP and T2-weighted images. In advanced cases with ductal obliterating lesions invading the surrounding pancreatic parenchyma, differentiation from pancreatic carcinoma may be difficult. In this situation, the prognosis also decreases and becomes similar to that of primary pancreatic carcinomas regardless of origin (1).
In patients with periampullary carcinomas of bile duct origin, the distal segment of the bile duct below the obstruction was also frequently seen on MRCP images; hence, three segments (the proximal and distal segments of the bile duct, and the main pancreatic duct) were depicted in the periampullary area (we call this the three-segment sign). The pancreatic duct is often normal until the tumor longitudinally infiltrates the ampullary portion or directly invades the pancreatic duct through the pancreatic parenchyma. This sign was noted in 20 cases overall; 15 of these were bile duct carcinomas, three were pancreatic carcinomas, and two were unclassified.
Periampullary duodenal carcinoma is a rare tumor, which abuts but spares or only partially involves the duodenal papillae and ampulla. In our series, only six cases satisfied this condition. Duodenal carcinomas may manifest as polypoid or fungating, ulcerative, and annular constrictive or infiltrative masses and are associated with lymphatic metastases in 22%–71% of cases (4). However, despite this relatively high frequency of nodal involvement, the 5-year survival rate approaches 40%–50% (1,18). When compared with other periampullary carcinomas, dilatation of the biliary or pancreatic duct was only modest or absent. Therefore, the ability of MR imaging to depict the mass depends on the size of the tumor and the degree of narrowing of the duodenal lumen. The presence of a certain amount of bright fluid and some degree of distention of the duodenal lumen are helpful in delineating the diseased region.
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Conclusions
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Although periampullary carcinomas share clinical presentations, anatomic locations, and therapeutic approaches, their long-term outcomes are varied. Accurate diagnosis and determination of the tumor origin may influence treatment planning and predicting prognosis. Pattern analysis of MRCP and conventional MR images, in terms of mass location and shape and the pattern of biliary and pancreatic ductal dilatation, may prove useful for differentiation of periampullary tumors.
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Footnotes
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Abbreviations: CBD = common bile duct,
GRE = gradient-echo,
MRCP = MR cholangiopancreatography,
SSFSE = single-shot fast spin-echo
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