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EDUCATION EXHIBIT |
Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC, Department of Radiology and Nuclear Medicine,, Uniformed Services University of the Health Sciences, Bethesda, Md
Benign epithelial tumors of the bile duct are uncommon and have been classified traditionally as adenomas, cystadenomas, or papillomatosis (1). Benign adenomas are polypoid lesions with tubu-lar, papillary, or tubulopapillary architecture. Cystadenomas are multilocular cystic neoplasms belonging to a unique group of tumors that may arise in the liver, gallbladder, extrahepatic bile ducts, pancreas, ovaries, or retroperitoneum. They are commonly referred to as mucinous cystic neoplasms. Mucinous cystadenomas are well recognized as having malignant potential. Papillomatosis is a very rare disorder characterized by multiple recurring papillary adenomas of the biliary tract. Many authors regard papillomatosis as a form of low-grade intraductal carcinoma because dysplasia is frequently present and because it is difficult to distinguish papillomatosis from papillary adenocarcinoma (1,2). Malignant transformation occurs less frequently in solitary papillary adenomas.
Abundant mucin production in solitary papillomas, papillomatosis, and papillary adenocarcinomas of the bile ducts recently has led many authors to report on the clinical and pathologic similarity of these lesions to intraductal papillary mucinous neoplasms of the pancreatic ducts (37). In this issue of RadioGraphics, Lim et al (8) further elaborate on their recently published experience (9) and describe the spectrum of clinical, pathologic, and imaging findings in 15 patients with intraductal papillary mucinous tumors of the bile ducts. They superbly illustrate the cross-sectional imaging and cholangiographic features of these unusual biliary tumors and point out the similarities between these tumors and intraductal papillary mucinous neoplasms of the pancreas.
Because few cases of mucin-producing papillary neoplasms of the bile ducts have been reported in the literature, the incidence and epidemiology are unknown. In my experience and that of my colleagues at the Armed Forces Institute of Pathology, mucin-producing papillary neoplasms of the bile ducts are exceedingly rare, compared with intraductal papillary mucinous neoplasms of the pancreas. Clearly, the clinical and radiologic hallmark of both lesion types is excessive mucin production with resultant focal or diffuse ductal dilatation, intraductal tumor nodules, mucin plugs, ductal obstruction, and inflammatory changes. Mounting evidence in the medical literature suggests that all of these tumors are part of a broad morphologic spectrum of intraductal papillary neoplasia (mucinous and nonmucinous) that may occur throughout the biliary and pancreatic ductal system. More study is needed to confirm and validate the clinical, imaging, and pathologic criteria for a diagnosis of intraductal papillary neoplasm of the bile ducts and to establish its identity with regard to its pancreatic counterparts.
In conclusion, the present article by Lim et al (8) gives us preliminary insight into the radiologic and pathologic features of mucin-secreting intraductal neoplasia of the bile ducts and provides a stimulus for further investigation into this rare group of neoplasms.
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