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(Radiographics. 2001;21:337-340.)
© RSNA, 2001


EDUCATION EXHIBIT

Invited Commentary • Author's Response

Angela D. Levy, LTC, MC, USA

Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC, Department of Radiology and Nuclear Medicine, Uniformed University School of Health Science, Bethesda, Md

Pablo R. Ros, MD, MPH

Department of Radiology, Brigham and Women’s Hospital and Harvard Medical School, Boston, Mass


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IPMT of the pancreas is an uncommon distinctive tumor that was originally described in the radiology and pathology literature in the late 1980s (1)–(4). Throughout the literature, a variety of names have been applied to this tumor, including intraductal papilloma, papillary adenoma, villous adenoma, diffuse intraductal papillary adenocarcinoma, diffuse villous carcinoma of the duct of Wirsung, carcinoma in situ of the pancreas, diffuse papillomatosis, carcinoma in situ, multiple primitive endoluminal tumors of the main pancreatic duct, intraductal mucin-hypersecreting neoplasm, ductectatic type of pancreatic ductal carcinoma, mucinous ductal ectasia, mucin-producing tumor (or carcinoma), and mucus-hypersecreting tumor (5).

The variable nomenclature for this tumor has led to considerable diagnostic confusion and is indicative of its diverse radiologic and histologic manifestations. The distribution of the tumor can be local or diffuse; it may involve the main pancreatic duct or branches, and histologically, it ranges from benign to malignant. The past nomenclature can also be confused with the more common terms "ductal adenocarcinoma of the pancreas" and "mucinous cystic neoplasm." The correct diagnosis of this tumor is prognostically significant because IPMT has a more favorable prognosis in comparison with ductal adenocarcinoma of the pancreas.

IPMT typically manifests in patients in the 7th decade of life. Symptoms may include vague abdominal pain, nausea, diarrhea, steatorrhea, and jaundice. Pancreatitis or pancreatitis-like symptoms may be present if there is complete or partial duct obstruction. Some patients may be asymptomatic, particularly if the tumor is in the tail of the pancreas. IPMT is more frequently found in the head or body of the pancreas and is more common in men (1),(2),(6),(7). Histologically, there is intraductal epithelial proliferation of mucin-producing cuboidal or tall columnar cells. The abundant mucin production creates ductal dilatation.

The cells are in a papillary or cribriform configuration in ectatic or cystically dilated main or branch pancreatic ducts. There may be varying degrees of cytoarchitectural atypia, comedo-type necrosis, and sialomucin production (7),(8). Foci of hyperplasia (with or without atypia or dysplasia), carcinoma in situ, and invasive carcinoma may be present in a single tumor. Thus, the degree of cytoarchitectural atypia may vary from case to case and in the same tumor (5). IPMT is not clearly classified as benign or malignant. Therefore, some authors have proposed that all of these tumors should be considered to have low-grade malignant potential (7).

In this issue of RadioGraphics, Lim et al illustrate the radiologic spectrum of IPMT, adding to the increased interest in and awareness of this entity. To our knowledge, they are only the second group of investigators to acknowledge and display the spectrum of radiologic appearances. Lim et al emphasize, as have others in the recent literature (1),(9)–(11), the limitations of cross-sectional imaging in the diagnosis of IPMT. Demonstration at ERCP of communication between a cystic lesion and the pancreatic duct or filling defects in the main pancreatic duct or side branch have been described as the most specific finding. There is mounting evidence in the literature, however, that the diagnosis can be made with thin-section CT or MRCP (9),(12)–(15).

When the findings of a bulging papilla, papillary projections in the ducts, or communication of the main pancreatic duct with a cystic lesion are present at CT or MRCP, the diagnosis can be made without invasive ERCP. Therefore, we would like to diverge from the opinion of Lim et al that ERCP is the procedure of choice for the diagnosis of IPMT. ERCP should be used in cases where the diagnosis is not clear at imaging.

Lim et al describe three morphologic patterns of IPMT: branch duct type, main duct type, and combined type. It should be noted that the main duct type may be focal or diffuse, and the branch duct type may have cystic side branches or dilated side branches (5),(16). Familiarity with the varied appearances and patterns of IPMT is crucial to its radiologic diagnosis (Figure).



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Figure 1. Diagrams illustrate four patterns of duct dilatation of IPMT of the pancreas: A, focal dilatation of the main pancreatic duct; B, diffuse dilatation of the main pancreatic duct, which may involve side branches; C, cystic side branch duct; and D, dilated side branch duct. (Adapted, with permission, from references 15 and 16.)

 
In summary, IPMT is an uncommon pancreatic neoplasm that has had confusing nomenclature throughout the medical literature and should be considered to have low-grade malignant potential preoperatively. Lim et al illustrate the varied morphologic appearances of IPMT and its spectrum of imaging findings. Noninvasive thin-section CT and MRCP images should be carefully evaluated for the specific findings of a bulging papilla, intraductal papillary projections, or communication between the main pancreatic duct and a cystic lesion.


    Footnotes
 
The opinions and assertion contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Departments of the Army or Defense.

The opinions and assertion contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Departments of the Army or Defense.


    References
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 Commentary
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 Response
 References 
 

  1. Itai Y, Kokubo T, Atomi Y, Kuroda A, Haraguchi Y, Terano A. Mucin-hypersecreting carcinoma of the pancreas. Radiology 1987; 165:51-55.[Abstract/Free Full Text]
  2. Itai Y, Ohhashi K, Nagai H, et al. "Ductectatic" mucinous cystadenoma and cystadenocarcinoma of the pancreas. Radiology 1986; 161:697-700.[Abstract/Free Full Text]
  3. Morohoshi T, Kanda M, Asanuma K, Kloppel G. Intraductal papillary neoplasms of the pancreas: a clinicopathologic study of six patients. Cancer 1989; 64:1329-1335.[Medline]
  4. Agostini S, Choux R, Payan MJ, Sastre B, Sahel J, Clement JP. Mucinous pancreatic duct ectasia in the body of the pancreas. Radiology 1989; 170:815-816.[Abstract/Free Full Text]
  5. Solacia E, Capella C, Kloppel G. Tumors of the pancreas. Atlas of tumor pathology, fasc 20, ser 3. Washington, DC: Armed Forces Institute of Pathology, 1997.
  6. Fukushima N, Mukai K. Pancreatic neoplasms with abundant mucus production: emphasis on intra-ductal papillary-mucinous tumors and mucinous cystic tumors. Adv Anat Pathol 1999; 6:65-77.[Medline]
  7. Paal E, Thompson LD, Przygodzki RM, Bratthauer GL, Heffess CS. A clinicopathologic and immunohistochemical study of 22 intraductal papillary mucinous neoplasms of the pancreas, with a review of the literature. Mod Pathol 1999; 12:518-528.[Medline]
  8. Kench JG, Eckstein RP, Smith RC. Intraductal papillary-mucinous neoplasm of the pancreas: a report of five cases with immunohistochemical findings. Pathology 1997; 29:7-11.[Medline]
  9. Fukukura Y, Fujiyoshi F, Sasaki M, Inoue H, Yonezawa S, Nakajo M. Intraductal papillary mucinous tumors of the pancreas: thin-section helical CT findings. AJR Am J Roentgenol 2000; 174:441-447.[Abstract/Free Full Text]
  10. McDowell RK, Gazelle GS, Murphy BL, et al. Mucinous ductal ectasia of the pancreas. J Comput Assist Tomogr 1997; 21:383-388.[Medline]
  11. Procacci C, Graziani R, Bicego E, et al. Intraductal mucin-producing tumors of the pancreas: imaging findings. Radiology 1996; 198:249-257.[Abstract/Free Full Text]
  12. Itoh S, Ishiguchi T, Ishigaki T, Sakuma S, Maruyama K, Senda K. Mucin-producing pancreatic tumor: CT findings and histopathologic correlation. Radiology 1992; 183:81-86.[Abstract/Free Full Text]
  13. Koito K, Namieno T, Ichimura T, et al. Mucin-producing pancreatic tumors: comparison of MR cholangiopancreatography with endoscopic retrograde cholangiopancreatography. Radiology 1998; 208:231-237.[Abstract/Free Full Text]
  14. Fukukura Y, Fujiyoshi F, Sasaki M, et al. HASTE MR cholangiopancreatography in the evaluation of intraductal papillary-mucinous tumors of the pancreas. J Comput Assist Tomogr 1999; 23:301-305.[Medline]
  15. Procacci C, Megibow AJ, Carbognin G, et al. Intraductal papillary mucinous tumor of the pancreas: a pictorial essay. RadioGraphics 1999; 19:1447-1463.[Abstract/Free Full Text]
  16. Furukawa T, Takahashi T, Kobari M, Matsuno S. The mucus-hypersecreting tumor of the pancreas: development and extension visualized by three-dimensional computerized mapping. Cancer 1992; 70:1505-1513.[Medline]

Author’s Response

Jae Hoon Lim, MD

Department of Radiology, Samsung Medical Center, Seoul, Korea


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We appreciate the valuable comments of Drs Levy and Ros. They enhance our aim of describing radiologic findings of IPMT, adding further interest. We agree with their views on the general description of the disease including histopathologic findings.

Regarding the role of cross-sectional imaging such as CT and MRCP versus ERCP, they stressed the relative importance of noninvasive thin-section CT and MRCP in the demonstration of bulging papilla, papillary projections in the pancreatic duct, and communication of the cyst with the pancreatic duct.

Bulging papilla into the lumen of the duodenum are encountered not only in IPMT but also in choledochal cyst (choledochocele); therefore, the finding is not characteristic of IPMT. Intraluminal papillary projections or mass in the dilated main pancreatic duct or branch duct is also a very convincing sign of IPMT. These two findings can be well visualized with thin-section CT. When they are visualized in association with characteristic dilatation of the main pancreatic duct or branch ducts, diagnosis is confident. The frequency of bulging papilla in IPMT is not high, however, being encountered in 22% of cases (1). In my experience and that of others (1), an intraluminal papillary mass is usually small and flat and thus difficult to detect.

Communication of the main pancreatic duct with a cystic lesion, although not conclusive, is an important finding in establishing the diagnosis of IPMT. Theoretically and practically, however, direct communication of a cystic lesion and the pancreatic duct is impossible to demonstrate with CT and MRCP. A cyst abutting or close to the pancreatic duct may look like a communication with the pancreatic duct at CT and MRCP. Mere contact of two lumina or cystic spaces at cross-sectional imaging is often considered communication in the radiology literature (1)–(5), but in fact, that is not true. We have several cases of noncommunicating neoplastic cysts or pseudocysts that we thought communicated with the pancreatic duct at thin-section (3 mm) CT or MRCP. Furthermore, communication with the pancreatic duct is not specific for IPMT because mucinous cystic neoplasm and pseudocyst frequently communicate with the pancreatic duct.

Therefore, ERCP is the most important modality, not only in the demonstration of intraductal tumor and communication of a cystic lesion with the typically dilated pancreatic duct for diagnosis, but also in the delineation of pathologic anatomy of pancreatic ductal dilatation for the planning of surgical resection. Additional crucial information available at ERCP is direct visualization of bulging papilla and demonstration of mucus protruding through the patulous papilla of Vater.

Morphologic patterns illustrated by Drs Levy and Ros are very informative. They classified the main duct type of IPMT as diffuse or focal and branch duct type as cystic or dilated side branches. In my opinion, cystic or simple dilatations of the side branches may be a matter of descriptive terms rather than different types.


    References 
 Top
 Commentary
 References
 Response
 References 
 

  1. Fukukura Y, Fujiyoshi F, Sasaki M, Inoue H, Yonezawa S, Nakajo M. Intraductal papillary mucinous tumors of the pancreas: thin-section helical CT findings. AJR Am J Roentgenol 2000; 174:441-447.
  2. Itoh S, Ishiguchi T, Ishigaki T, Sakuma S, Maruyama K, Senda K. Mucin-producing pancreatic tumor: CT findings and histopathologic correlation. Radiology 1992; 183:81-86.
  3. Koito K, Namieno T, Ichimura T, et al. Mucin-producing pancreatic tumors: comparison of MR cholangiopancreatography with endoscopic retrograde cholangiopancreatography. Radiology 1998; 208:231-237.
  4. Fukukura Y, Fujiyoshi F, Sasaki M, et al. HASTE MR cholangiopancreatography in the evaluation of intraductal papillary-mucinous tumors of the pancreas. J Comput Assist Tomogr 1999; 23:301-305.
  5. Onaya H, Itai Y, Niitsu M, Chiba T, Michishita N, Saida Y. Ductectatic mucinous cystic neoplasms of the pancreas: evaluation with MR cholangiopancreatography. AJR Am J Roentgenol 1998; 171:171-177.[Abstract/Free Full Text]

Related Article

Radiologic Spectrum of Intraductal Papillary Mucinous Tumor of the Pancreas
Jae Hoon Lim, Gina Lee, and Young Lyun Oh
RadioGraphics 2001 21: 323-337. [Abstract] [Full Text] [PDF]




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