Mucin-hypersecreting bile duct neoplasm characterized by clinicopathological resemblance to intraductal papillary mucinous neoplasm (IPMN) of the pancreas
© Yamashita et al; licensee BioMed Central Ltd. 2007
Received: 05 May 2007
Accepted: 28 August 2007
Published: 28 August 2007
Although intraductal papillary mucinous neoplasm (IPMN) of the pancreas is acceptable as a distinct disease entity, the concept of mucin-secreting biliary tumors has not been fully established.
We describe herein a case of mucin secreting biliary neoplasm. Imaging revealed a cystic lesion 2 cm in diameter at the left lateral segment of the liver. Duodenal endoscopy revealed mucin secretion through an enlarged papilla of Vater. On the cholangiogram, the cystic lesion communicated with bile duct, and large filling defects caused by mucin were observed in the dilated common bile duct. This lesion was diagnosed as a mucin-secreting bile duct tumor. Left and caudate lobectomy of the liver with extrahepatic bile duct resection and reconstruction was performed according to the possibility of the tumor's malignant behavior. Histological examination of the specimen revealed biliary cystic wall was covered by micropapillary neoplastic epithelium with mucin secretion lacking stromal invasion nor ovarian-like stroma. The patient has remained well with no evidence of recurrence for 38 months since her operation.
It is only recently that the term "intraductal papillary mucinous neoplasm (IPMN)," which is accepted as a distinct disease entity of the pancreas, has begun to be used for mucin-secreting bile duct tumor. This case also seemed to be intraductal papillary neoplasm with prominent cystic dilatation of the bile duct.
Some cases of papillary adenocarcinoma, cystadenocarcinoma, cystadenoma, or papillomatosis have been described as mucin-secreting biliary tumors [1–3]. Although intraductal papillary mucinous neoplasm (IPMN) of the pancreas is acceptable as a distinct disease entity, [4–6] the concept of mucin-secreting biliary tumors has not been established [7, 8]. Kim et al., first used the term "IPMN" for nine cases of mucin-hypersecreting bile duct tumor in 2000 , and the condition has been described with increasing frequency in recent years [7, 10–12]. We herein report a case of mucin-secreting biliary neoplasm that is clinicopathologically similar to IPMN of the pancreas.
A 71-year-old Japanese woman was referred to our hospital for evaluation of her elevation of serum gamma glutamyl transferase levels to 135 IU/L (reference value 16–73 IU/L), which were incidentally found in a biochemical test in medical health check. She had no symptoms of epigastralgia or jaundice. Her physical examination and past medical history was unremarkable. She did not smoke or drink. The remainder of her serum chemistries, complete blood count, and coagulation profile were all normal. Serum levels of tumor marker such as carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) were within normal limits.
While the concept of mucin-producing pancreatic tumors is well-recognized, the concept of a mucin-secreting biliary tumor has not been well-established. Considering the shared embryonic origin of the two duct system , mucin-secreting bile duct tumor, like an IPMN of the pancreas, may theoretically also exist. Based on the imaging for intraductal papillary tumors of the bile duct, mucin component was detected as filling defect at cholangiography and the papillary tumor was detected as polypoid mass in the dilated biliary tree as well as IPMN of pancreas [1, 12]. Zen et al., summarized pathological similarities and differences between IPN of bile duct and IPMN of pancreas . Similar findings were intraductal papillary proliferation with fibrovascular core, occasional mucin hypersecretion, most of mucin profiles, and patient outcome, while dissimilar findings were pointed out to be the incidence of immunohistochemical cytokeratin 20 expression, the percentage of gastric-type tumors, and the frequency of mucin hypersecretion. IPMN of the pancreas is a grossly visible, non-invasive, mucin-producing, predominantly papillary epithelial neoplasm with varying degree of cellular atypia, arising from pancreastic ducts with duct dilatation . A mass component of papillary proliferation commonly seen in IPMN of pancreas was absent and micropapillary proliferation was predominant in our case, but the histological features of cystic dilated duct with mucin hypersecreation, and moderate cellular atypia were resemble with that of IPMN of pancreas. On the other hand, hepatobiliary cystadenoma/adenocarcinoma in middle-aged women have been reported to have ovarian-like stroma [14, 15] and a large number of these cases would enter the disease entity of a mucinous cystic neoplasm (MCN) of the pancreas . Our case did not have ovarian-like stroma in the cystic wall. These findings and mucin profiles suggest that our biliary lesion resemble with cystic variant of intraductal papillary neoplasm of the bile duct, proposed by Zen et al., . Lim JH.,  have summarized the characteristics observed in the imaging of IPMN of the bile duct from 15 cases, and our case is compatible with these descriptions at an aneurysmal type. Also in our case, ERC or PTC instead of US, CT, or MRI made it possible to detect mucin. We therefore consider that ERC or PTC is necessary to diagnose IPMN of the bile duct.
According to the adenoma-carcinoma sequences in the IPMN of the pancreas  we treated our case according to the malignant behavior. Percutaneous transhepatic cholangioscopy (PTCS) was recommended for the accurate diagnosis of IPMN of the bile duct, [1, 9, 11] but we did not perform PTCS because of its invasiveness, the time required to complete the procedure, and the risk of malignant seeding of the tract . Lim JH et al.,  have also reported that 7 of 15 (47.3%) patients with IPMN of the bile duct had multiple IPMN in their resected specimens. Therefore, intraoperative cholangioscopy and frozen histology should have been performed in our case. If frozen histology had revealed no malignancy in this case, more limited resection such as left lobectomy of the liver without caudate lobectomy of the liver and extrahepatic bile duct resection could have been performed.
If more cases of IPN of the bile duct are reported, consensus could be achieved regarding the optimal and least invasive preoperative evaluation and treatment plans such as indications for surgery and the extent of resection.
It is only recently that the term "intraductal papillary mucinous neoplasm (IPMN)," which is accepted as a distinct disease entity of the pancreas, has begun to be used as "intraductal papillary neoplasm of the bile duct (IPN-B)" for mucin-secreting bile duct tumor. If more cases of IPN of the bile duct are reported, consensus could be achieved regarding the optimal and least invasive preoperative evaluation and treatment plans.
Written consent was obtained from the patient for publication of this case report.
- Sakamoto E, Hayakawa N, Kamiya J, Kondo S, Nagino M, Kanai M, Miyachi M, Uesaka K, Nimura Y: Treatment strategy for mucin-producing intrahepatic cholangiocarcinoma: Value of percutaneous transhepatic biliary drainage and cholangioscopy. World J Surg. 1999, 23: 1038-1044. 10.1007/s002689900620.View ArticlePubMedGoogle Scholar
- Styne P, Warren GH, Kumpe DA, Halgrimson C, Kern F: Obstructive cholangitis secondary to mucus secreted by a solitary papillary bile duct tumor. Gastroenterology. 1986, 90: 748-753.PubMedGoogle Scholar
- Wang YL, Lee SD, Lai KH, Wang SS, Lo KJ: Primary biliary cystic tumors of the liver. Am J Gastroenterol. 1993, 88: 599-603.PubMedGoogle Scholar
- Fukushima N, Mukai K: Pancreatic neoplasms with abundant mucus production: emphasis on intraductal papillary-mucinous tumors and mucinous cystic tumors. Adv Anat Pathol. 1999, 6: 65-77. 10.1097/00125480-199903000-00001.View ArticlePubMedGoogle Scholar
- Longnecker DS, Hruban RH, Adler G, Kloppel G: Intraductal papillary-mucinous neoplasm of the pancreas; World Health Organization International Histological Classification of Tumors, Pathology and Genetics of Tumours of the Digestive System. 2000, Lyon, France: IARC Press, 237-240.Google Scholar
- Hruban RH, Takaori K, Klimstra DS, Adsay NV, Albores-Saaverdra J, Biankin AV, Biankin SA, Compton C, Fukushima N, Furukawa T, Goggins M, Kato Y, Kloppel G, Longnecker DS, Luttges J, Maitra A, Offerhaus GJ, Shimizu M, Yonezawa S: An illustrated consensus on the classification of pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasms. Am J Surg Pathol. 2004, 28: 977-987. 10.1097/01.pas.0000126675.59108.80.View ArticlePubMedGoogle Scholar
- Shibahara H, Tamada S, Goto M, Oda K, Nagino M, Nagasaka T, Batra SK, Hollingsworth MA, Imai K, Nimura Y, Yonezawa S: Pathologic features of mucin-producing bile duct tumors: two histopathologic categories as counterparts of pancreatic intraductal papillary-mucinous neoplasms. Am J Surg Pathol. 2004, 28: 327-338. 10.1097/00000478-200403000-00005.View ArticlePubMedGoogle Scholar
- Zen Y, Fujii T, Itatsu K, Nakamura K, Minato H, Kasashima S, Kurumaya H, Katayanagi K, Kawashima A, Masuda S, Niwa H, Mitsui T, Asada Y, Miura S, Ohta T, Nakanuma Y: Biliary papillary tumors share pathological features with intraductal papillary mucinous neoplasm of the pancreas. Hepatology. 2006, 44: 1333-1343. 10.1002/hep.21387.View ArticlePubMedGoogle Scholar
- Kim HJ, Kim MH, Lee SK, Yoo KS, Park ET, Lim BC, Park HJ, Myung SJ, Seo DW, Min YI: Mucin-hypersecreting bile duct tumor characterized by a striking homology with an intraductal papillary mucinous tumor (IPMT) of the pancreas. Endoscopy. 2000, 32: 389-393. 10.1055/s-2000-8996.View ArticlePubMedGoogle Scholar
- Oshikiri T, Kashimura N, Katanuma A, Maguchi H, Shinohara T, Shimizu M, Kondo S, Katoh H: Mucin-secreting bile duct adenoma-clinicopatological resemblance to intraductal papillary mucinous tumor of the pancreas. Dig Surg. 2002, 19: 324-327. 10.1159/000064570.View ArticlePubMedGoogle Scholar
- Somogri L, Dimashkieh H, Weber FL, Buell J: Biliary intraductal papillary mucinous tumor: diagnosis and localization by endoscopic retrograde cholangioscopy. Gastrointest Endosc. 2003, 57: 620-622. 10.1067/mge.2003.157.View ArticleGoogle Scholar
- Lim JH, Yoo KH, Kim SH, Kim HY, Lim HK, Song SY, Nam KJ: Intraductal papillary mucinous tumor of the bile ducts. RadioGraphics. 2004, 24: 53-67. 10.1148/rg.241035002.View ArticlePubMedGoogle Scholar
- Sadler TW: Langman's Medical Embryology. 2006, Baltimore, Williams&Wilkins, 10Google Scholar
- Buetow PC, Buck JL, Pantongrag-Brown L, Ros PR, Devaney K, Goodman ZD, Cruess DF: Biliary cystadenoma and cystadenocarcinoma: clinical-imaging-pathologic correlations with emphasis on the importance of ovarian stroma. Radiology. 1995, 196: 805-810.View ArticlePubMedGoogle Scholar
- Devaney K, Goodman ZD, Ishak KG: Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol. 1994, 18: 1078-1091.View ArticlePubMedGoogle Scholar
- Inagaki M, Maguchi M, Kino S: Mucin-producing tumors of the pancreas: clinicopathological features, surgical treatment, and outcome. J Hepatobiliary Pancreat Surg. 1999, 6: 281-285. 10.1007/s005340050119.View ArticlePubMedGoogle Scholar
- Zen Y, Fujii T, Itatsu K, Nakamura K, Konishi F, Masuda S, Mitsui T, Asada Y, Miura S, Miyayama S, Uehara T, Katsuyama T, Ohta T, Minato H, Nakanuma Y: Biliary cystic tumors with bile duct communication: a cystic variant of intraductal papillary neoplasm of the bile duct. Mod Pathol. 2006, 19: 1243-1254. 10.1038/modpathol.3800643.View ArticlePubMedGoogle Scholar
- Obara T, Maguchi H, Saitoh Y: Mucin-producing tumor of the pancreas: Natural history and serial pancreatogram changes. Am J Gastroenterol. 1993, 88: 564-569.PubMedGoogle Scholar
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