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Making Sense of Mucin-Producing Pancreatic Tumors

John R. Grogan1, Kia Saeian2, Andrew J. Taylor1,3, Francisco Quiroz1, Michael J. Demeure4 and Richard A. Komorowski5

1 Department of Radiology, Medical College of Wisconsin, 8701 Watertown Plank Rd., Milwaukee, WI 53226.
2 Department of Medicine, Medical College of Wisconsin, Milwaukee, WI 53226.
3 Present address: Department of Radiology, Box 3252, University of Wisconsin Medical School, E3/311 Clinical Science Center, 600 Highland Ave., Madison, WI 53792-3252.
4 Department of Surgery, Medical College of Wisconsin, Milwaukee, WI 53226.
5 Department of Pathology, Medical College of Wisconsin, Milwaukee, WI 53226.



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Fig. 1. Side branch intraductal papillary mucinous neoplasm. Drawing shows abnormal cells in uncinate side branches that produce large enough quantity of mucus (yellow) to distend side branch system. Mucus can spill over to enlarge adjacent main pancreatic duct. Note that papillary orifice gapes with extruding mucus. Abnormal cells that are not visible may be hyperplastic lining (blue). Subtle elevated area or polypoid projection (purple) can occasionally be seen separate from mobile mucus.

 


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Fig. 2. 34-year-old man with pancreatitis who presented for endoscopic retrograde cholangiopancreatography (ERCP). Endoscopic image shows patulous papillary orifice (arrow) with mucus bulging into lumen. Note ERCP cannula (asterisk).

 


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Fig. 3. Main duct intraductal papillary mucinous neoplasm. This schematic illustrates marked main pancreatic duct distention from mucus (yellow). Pancreatic parenchyma atrophies. As in side branch intraductal papillary mucinous neoplasm, abnormal mucus-producing cells are usually subtle in appearance if seen at all. Blue = hyperplastic cells, purple = intraluminal excrescences.

 


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Fig. 4. Mucinous cystic neoplasm. Schematic shows mass emanating from pancreas without connection to duct system. Capsule is formed from outer fibrous sheath (stippled) and inner ovarian stroma (pink). Lining may be hyperplastic (blue) or develop intraluminal projections such as polyps (purple) or septa (white). There may be areas of normal columnar epithelia, which can result in false-negative findings at biopsy if more aggressive epithelia present are not sampled. Yellow = mucus.

 


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Fig. 5A. Side branch intraductal papillary mucinous neoplasm in 46-year-old man with abdominal pain and pancreatitis. CT scan obtained through pancreatic head shows numerous small "cysts" (arrows).

 


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Fig. 5B. Side branch intraductal papillary mucinous neoplasm in 46-year-old man with abdominal pain and pancreatitis. CT scan obtained 10 mm caudad to A shows cyst is actually composed of dilated main pancreatic duct (d) and enlarged uncinate side branch (b), which forms curvilinear tube.

 


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Fig. 6. Main duct intraductal papillary mucinous neoplasm in 66-year-old male renal transplant patient with abdominal pain and recent onset of diabetes. Unenhanced CT scan through pancreatic body shows diffusely dilated main pancreatic duct (arrows) with severe parenchymal atrophy.

 


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Fig. 7. Mucinous cystic neoplasm in 63-year-old woman with pancreatic mass found at workup for vague left upper quadrant fullness. CT scan shows complex, multilocular mass involves body and tail. At surgery, this mucinous cystic neoplasm was found to be malignant with metastatic disease to liver.

 


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Fig. 8A. Endoscopic retrograde pancreatograms show side branch intraductal papillary mucinous neoplasm in this 38-year-old man presenting with recurrent bouts of epigastric pain. Early during injection of contrast material, filling defects are seen in main pancreatic duct (straight arrows). Dilated uncinate side branch is partially filled with mucus (curved arrow), making it difficult to see.

 


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Fig. 8B. Endoscopic retrograde pancreatograms show side branch intraductal papillary mucinous neoplasm in this 38-year-old man presenting with recurrent bouts of epigastric pain. After most of mucus has been removed using retrieval balloon, abnormal side branch (arrowhead) can be readily seen.

 


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Fig. 9A. Endoscopic retrograde pancreatogram shows main duct pancreatic intraductal papillary mucinous neoplasm in this 66-year-old renal transplant patient with abdominal pain and diabetes (same patient as in Fig. 6). Early during injection of contrast material, there is massively dilated main pancreatic duct with diffuse filling defects.

 


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Fig. 9B. Endoscopic retrograde pancreatogram shows main duct pancreatic intraductal papillary mucinous neoplasm in this 66-year-old renal transplant patient with abdominal pain and diabetes (same patient as in Fig. 6). With removal of mucus, large polypoid mass (arrow) is revealed. Brushings were obtained at this time.

 


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Fig. 10. 56-year-old woman with pancreatitis is shown to have calcification associated with an intraductal papillary mucinous neoplasm. Endoscopic retrograde pancreatogram shows calcification (arrowhead) in main pancreatic duct just above sphincteric segment. This large calcification straddles junction of main duct and uncinate side branch, which was later filled.

 


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Fig. 11. 34-year-old woman with recurrent bouts of pancreatitis. Endoscopic retrograde pancreatogram shows dilated side branch (arrowhead), but throughout this and subsequent examinations, no filling defects were discovered. No mucin was returned on balloon sweeps. However, because of side branch ectasia, diagnosis of intraductal papillary mucinous neoplasm was made and subsequently proved at surgery.

 


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Fig. 12A. 54-year-old man with recurrent pancreatitis who underwent both CT and MR imaging for what was eventually found at surgery to be side branch intraductal papillary mucinous neoplasm. CT scan shows complex cystic mass (arrow) in pancreatic head.

 


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Fig. 12B. 54-year-old man with recurrent pancreatitis who underwent both CT and MR imaging for what was eventually found at surgery to be side branch intraductal papillary mucinous neoplasm. Gadolinium-enhanced MR image shows better than CT scan individual ectatic side branches (arrowhead) forming tubes within uncinate process.

 


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Fig. 13A Incidental mass found on lumbar spine MR image in this 80-year-old woman with lower back pain. Diagnosis of mucinous cystic neoplasm was made on basis of imaging characteristics in association with patient's sex, age, and lack of presenting complaints. Large mass in pancreatic tail can be seen on CT scan. Contrast material could not be given because of renal failure. Mass appears to be unilocular. Notice calcification in wall (arrow).

 


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Fig. 13B. Incidental mass found on lumbar spine MR image in this 80-year-old woman with lower back pain. Diagnosis of mucinous cystic neoplasm was made on basis of imaging characteristics in association with patient's sex, age, and lack of presenting complaints. T2-weighted MR image better shows complex septa not seen on CT scan.

 


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Fig. 14. 71-year-old man who presented for endoscopic sonography after detection of incidental pancreatic mass found during CT workup (not shown) for biopsy-proven hepatocellular carcinoma. Endoscopic sonogram shows pancreatic mass (arrows) to be complex with thick septa and nodules. Biopsy sample was obtained at this time. Malignant mucinous tumor, intraductal papillary mucinous neoplasm, was diagnosed at pathologic examination. Asterisk = needle.

 


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Fig. 15A. Chronic calcific pancreatitis simulates intraductal papillary mucinous neoplasm at CT in this 57-year-old man with recurrent bouts of pancreatitis. Pancreatic CT performed in 1991 (not shown) showed only subtle main pancreatic duct dilatation. Six years later, CT shows marked change. CT scan obtained through body and tail of pancreas shows diffuse dilatation of main pancreatic duct (arrow) with calcifications (arrowheads).

 


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Fig. 15B. Chronic calcific pancreatitis simulates intraductal papillary mucinous neoplasm at CT in this 57-year-old man with recurrent bouts of pancreatitis. Pancreatic CT performed in 1991 (not shown) showed only subtle main pancreatic duct dilatation. Six years later, CT shows marked change. CT scan obtained just caudad to A shows complex cystic mass related to uncinate is present (arrow). Patient had only changes of chronic calcific pancreatitis at surgical resection without abnormal epithelial cells.

 


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Fig. 16. Pancreatic carcinoma simulates mucinous cystic neoplasm in 63-year-old woman who presented with left upper quadrant pain and no history of alcohol abuse. Contrast-enhanced CT scan revealed complex mass (straight arrows) in pancreatic tail. There is extension of this mass (arrowhead) into spleen, with splenic infarction and thrombosed splenic vein. Adjacent pancreas (curved arrow) is abnormally low in attenuation. At surgery, pancreatic ductal carcinoma was found with extensive local spread and associated pseudocyst.

 


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Fig. 17. Pancreatic ductal carcinoma simulates intraductal papillary mucinous neoplasm in this 57-year-old man with abdominal distention and jaundice. CT scan reveals marked dilatation of main pancreatic duct (arrow). This dilatation with parenchymal atrophy could simulate main duct intraductal papillary mucinous neoplasm. However, there is dilatation of biliary tree as well. Biopsy of low-attenuation area within uncinate process (not shown) revealed pancreatic ductal carcinoma.

 


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Fig. 18. Intraductal pancreatic ductal adenocarcinoma simulates main duct intraductal papillary mucinous neoplasm in this 71-year-old man presenting with steatorrhea. Endoscopic retrograde pancreatogram shows nodular filling defect on initial injection of contrast material. On subsequent injection, this filling defect did not move. There is ductal dilatation upstream. At surgery, this was ductal pancreatic carcinoma with primarily intraductal growth.

 


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Fig. 19A. Islet cell tumor simulating mucinous cystic neoplasm. 38-year-old woman with pancreatic mass found during pregnancy. CT scan shows cystic mass (arrow) in pancreatic head. At surgery, this mass proved to be cystic islet cell tumor.

 


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Fig. 19B. Islet cell tumor simulating mucinous cystic neoplasm. 63-year-old man who presented with diarrhea. CT scan shows complex mass (arrow) involving tail of pancreas. At surgery, this mass was necrotic islet cell tumor of pancreas.

 


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Fig. 20. Necrotic pancreatic sarcoma mimics mucinous cystic neoplasm in this 58-year-old man with left upper quadrant pain. CT scan reveals large complex mass that could be interpreted as mucinous cystic neoplasm, but at surgery mass was shown to be sarcoma. Note metastasis to liver (arrows).

 


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Fig. 21. 16-year-old girl who presented with mid epigastric pain was found to have solid and pseudopapillary epithelial neoplasm, which could be confused with mucinous cystic neoplasm. CT scan shows complex mass (arrow) in pancreatic head. Even though this mass could simulate mucinous cystic neoplasm, age and sex of this patient would more likely indicate solid and pseudopapillary epithelial neoplasm, which was removed at surgery.

 


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Fig. 22. Multiloculated mass, incidentally found on this CT scan, in 51-year-old woman has appearance of a mucinous cystic neoplasm; however, at surgery, it proved to be serous cystadenoma.

 


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Fig. 23. 45-year-old woman who presented with epigastric pain. On CT scan, pancreatic head mass appears fairly well circumscribed and of water attenuation with small nodule in its wall (arrow). This mass proved to be lymphoepithelial cyst at surgery.

 


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Fig. 24A. 28-year-old woman with von Recklinghausen's disease who presented with complaints of abdominal pain related to pancreatitis. CT scan obtained through pancreatic body and tail reveals moderate dilatation of main pancreatic duct (arrow).

 


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Fig. 24B. 28-year-old woman with von Recklinghausen's disease who presented with complaints of abdominal pain related to pancreatitis. CT scan obtained slightly caudad to A shows pancreatic duct dilatation coursing toward papilla (arrowhead). At surgery, periampullary adenoma was selectively obstructing pancreatic duct at ampulla.

 

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