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  Table of Contents    
BRIEF COMMUNICATION  
Year : 2014  |  Volume : 57  |  Issue : 4  |  Page : 588-590
Biliary tract intraductal papillary mucinous neoplasm: A brief report and review of literature


1 Department of Surgical Gastroenterology, Government Medical College, Thiruvananthapuram, Kerala, India
2 Department of Pathology, Government Medical College, Thiruvananthapuram, Kerala, India

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Date of Web Publication11-Oct-2014
 

   Abstract 

Biliary Tract Intraductal Papillary Mucinous Neoplasm (BT-IPMN) is a very rare entity, gradually emerging into attention as sporadic cases are being reported worldwide. In this brief report we discuss about such an entity from our part of the world, based on a case from our institution. A 47-year-old female was referred to our department with jaundice, intermittent fever with chills and rigor of 6 weeks duration. Initial evaluation revealed obstructive jaundice with distended gall bladder. Imaging with ultrasonogram (USG) and magnetic resonance imaging (MRI) showed hugely dilated intra and extrahepatic biliary radicles with multiple and diffuse soft tissue lesions filling the common bile duct (CBD) extending to the ductal system of left lobe of liver. A side viewing endoscopy demonstrated mucin extruding from a prominent ampulla of Vater. The patient was managed successfully by left hepatectomy with pancreaticoduodenectomy (HPD). Gross pathological examination of the specimen showed marked dilatation of intra and extra hepatic bile ducts with multiple polypoidal lesions and plenty of mucin filling the entire biliary ductal system. Histopathology revealed predominantly intraductal papillary mucinous adenocarcinoma at the hilum extending to left bile duct with diffuse dysplastic changes throughout the biliary tree. Thus the clinical, radiological and pathological features of this lesion clearly fit into the diagnosis of BT-IPMN, which is slowly being established as a definite clinical entity with features much similar to its pancreatic counterpart.

Keywords: Biliary tract, intraductal papillary mucinous neoplasm, pancreas, papillary adenocarcinoma

How to cite this article:
Subhash R, Valiyaveettil IA, Natesh B, Raji L. Biliary tract intraductal papillary mucinous neoplasm: A brief report and review of literature . Indian J Pathol Microbiol 2014;57:588-90

How to cite this URL:
Subhash R, Valiyaveettil IA, Natesh B, Raji L. Biliary tract intraductal papillary mucinous neoplasm: A brief report and review of literature . Indian J Pathol Microbiol [serial online] 2014 [cited 2019 Nov 20];57:588-90. Available from: http://www.ijpmonline.org/text.asp?2014/57/4/588/142676



   Introduction Top


Sporadic case reports of BT-IPMN have brought more and more attention to this entity which was once regarded as a myth. [1],[2],[3] Since the 1960s, a variety of mucin secreting, papillary and/or cystic lesions of the intra and extra hepatic biliary tract have been recognized and reported worldwide. The clinical and pathological characteristics of these biliary neoplasms were closely similar to that of pancreatic intraductal papillary mucinous neoplasm (IPMN) and so known by the name BT-IPMN. [1],[4] Now BT-IPMN has been recognized as an entity with meaningful occurrence and can be diagnosed readily on histopathological examination using the criteria developed by the WHO. [4] Here we report a case of BT-IPMN from our part of the world.


   Case report Top


A 47-year-old female was referred to our department with the history of mild jaundice and intermittent fever with chills and rigor of 6 weeks duration. She had loss of appetite and significant loss of weight. She also gave a history of similar episodes of intermittent fever with chills and rigor for the last 1 year. She had no co-morbidities. Clinical examination showed mild icterus, hepatomegaly and a palpable gall bladder. On examination, other systems were within normal limits. A provisional clinical diagnosis of periampullary carcinoma was made and the patient was subjected to a detailed evaluation. Her blood examination showed hemoglobin 9.1g%, total leukocyte count 15100/ μL with predominantly neutrophils (84%), erythrocyte sedimentation rate 115 mm in the first hour, total bilirubin 4.0 mg/dL with direct bilirubin 2.6 mg/dL, total protein 7.9 gm/dL, albumin 3.0 gm/dL, aspartate aminotransferase 82 IU/L, alanine aminotransferase 94 IU/L and alkaline phosphatase 893 IU/L. Other blood parameters were normal. Estimation of the serum tumor markers were as follows; Carbohydrate Antigen 19-9 -236 u/ml, Carcino Embryonic Antigen- 6 ng/ml. Upper gastrointestinal endoscopy was normal. Side viewing endoscopy showed mucous discharge from a prominent but otherwise normal looking papilla. Initial USG revealed distended gall bladder with dilated CBD and intra hepatic biliary radicles predominantly on the left side. A mass lesion was also identified at the hilum of bile ducts extending down to the CBD. So an MRI of the abdomen with magnetic resonance cholangio pancreatogram (MRCP) was performed, which showed dilatation of the CBD, hepatic ducts and intra-hepatic biliary radicles with a lobulated lesion with altered signal intensity filling the left hepatic duct and CBD with diffuse enhancement [Figure 1]. Multiple smaller lesions with altered signal intensity were also noted filling the CBD. There was also thrombosis of the left portal vein. Imaging features were suggestive of diffuse cholangiocarcinoma polypoidal type.
Figure 1: MRI of the abdomen [(a) MRCP and (b) axial sections] showing dilated intra and extra hepatic biliary ducts and distended gall bladder with multiple altered signal intensity lesions filling the CBD and left hepatic duct

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In view of diffuse cholangiocarcinoma predominantly affecting left biliary ducts, we proceeded with left hepatectomy. Intra-operatively there was hypertrophy of the right lobe with relative atrophy of the left lobe and multiple nodular projections on the surface of the left lobe of liver. Gall bladder was distended and CBD was grossly dilated up to the lower end. There was also, thickening and nodularity felt inside the CBD distally upto peri ampullary region. No vascular infiltration was noted at hilum. There was no ascites and no evidence of peritoneal or omental dissemination. In view of these findings, left hepatectomy with en bloc excision of entire extrahepatic biliary tree was planned, which would naturally end up in pancreaticoduodenectomy as well. Thus a hepato-pancreaticoduodenectomy (HPD) was performed. Finally the entire specimen was removed en-bloc with the lymph nodes [Figure 2]a.

On sectioning the specimen on the side table, bile ducts were filled with mucoid material. There were polypoidal growths with long pedicle protruding out through the cut end of the bile duct [Figure 2]b, [Figure 2]c. Histopathology analysis of specimen showed atypical biliary epithelium proliferated in a papillary fashion, with thin fibro-vascular cores at multiple sites within the dilated bile ducts [Figure 3]a. Diffuse dysplastic changes of varying degree were observed in the rest of the areas of the ductal system [Figure 3]b. Ductal epithelium also demonstrated plenty of extra and intracellular mucin throughout the specimen [Figure 4]a. Histopathology of large polypoidal lesions arising near the hilum demonstrated well differentiated papillary mucinous adenocarcinoma with adjacent liver infiltration [Figure 4]b. Surprisingly, all lymph nodes showed only reactive changes. The pancreas was normal.
Figure 2: (a) Hepato-Pancreaticoduodenectomy gross specimen (b, c) specimen showing polypoidal lesions protruding from the cut opened bile ducts (arrows)

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Figure 3: Photomicrographs of the BT-IPMN. (a) H and E stain 400× light micrograph of papillary adenocarcinoma of biliary epithelium with thin fibrovascular core and mucin production (b) H and E stain 100× light micrograph of biliary epithelium showing diffuse dysplastic changes

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Figure 4: (a) H and E stain 400× light micrograph showing pools of mucin along with tumor cells (b) H and E stain 100× light micrograph demonstrating areas of papillary adenocarcinoma infiltrating adjacent
liver tissue


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Postoperative recovery of the patient was uneventful apart from a surgical site infection, which settled with daily dressing and culture based antibiotics. She was discharged on post-operative day 15. The patient remains well with no evidence of disease at one year follow-up.


   Discussion Top


BT-IPMN is an entity, which has been previously described as biliary papillomatosis, bile duct cystadenocarcinoma or mucin hypersecreting bile duct tumor. [1],[5],[6] In several aspects these lesions resemble IPMN of the pancreas. [2],[3] In both the organs, the neoplasms originate and proliferate within the ductal system and show mucin overproduction and have an association with invasive adenocarcinoma. [4] So now they are increasingly known by the name BT-IPMN.

The true incidence of BT- IPMN is unknown. A retrospective study showed 7% incidence of BT- IPMN among all biliary neoplasms resected during a period of 12 years using WHO histopathological criteria. [4] There are no definite diagnostic criteria for BT-IPMN so far. The WHO criteria for pancreatic IPMN are generally adopted for BT-IPMN as well, by various groups. [4],[7] International consensus guidelines define IPMN as a 'grossly visible, non-invasive, mucin-producing, predominantly papillary or, rarely flat, epithelial neoplasm'. [8] These guidelines were created in order to clearly delineate IPMN from pancreatic intraepithelial neoplasm (PanIN) which can be impossible to differentiate by microscopic evaluation of routine haematoxylin and eosin (H and E) stains only. In fact, the presence of a macroscopic intraluminal lesion and visible mucin on the surface of the tumor are the main characteristics differentiating IPMN from PanIN. [4] Similarly the diagnosis of BT-IPMN is also based on the presence of an intraluminal neoplasm with papillary cyto-architecture and mucin hypersecretion. [1],[3],[8] Because of mucin hypersecretion, affected bile ducts exhibit marked dilatation, and tumors are confined within the dilated part of the bile duct. Parts of the biliary tree not affected by neoplasia are also sometimes dilated. Thus BT-IPMN is pathologically defined by features like prominent intraductal papillary neoplasm with thin fibrovascular cores, frequent mucin hyper secretion, acquisition of gastrointestinal phenotypes, and common association with invasive mucinous adenocarcinoma. [1],[3],[4] At histopathologic analysis it may present as adenocarcinoma, adenoma, dysplasia or as a mixed variety. The frequency of invasive carcinoma in patients with resected BT-IPMN appears to be greater than in patients with resected pancreatic IPMN. In a study, 83% of patients with resected BT-IPMN had invasive carcinoma compared with only 30% in resected pancreatic IPMN. [4]

Recently a pathological classification of BT-IPMN has been introduced after retrospective analysis of resected specimens. [9] It is classified into four types; Type 1-lined by biliary epithelium of low-grade dysplasia; type 2-lined by biliary epithelium of high-grade dysplasia; type 3-lined by in situ and micro invasive adenocarcinoma; and type 4-lined by papillary lesions with stromal invasion of adenocarcinoma. A clinical classification of BT-IPMN based on the cholangiographic pattern was also suggested. [6] There are three types with subtypes; type 1A-hepatolithiasis with biliary stricture; type 1B-fusiform biliary tree with amorphous floating filling defects in absence of discernible neoplasia; type 1C- disproportional biliary dilatation in absence of discernible neoplasia; type IIA-intrahepatic polypoid or cystic neoplasia; type IIB- intrahepatic polypoid or cystic neoplasia extending to the extrahepatic bile duct; type IIIA-type 1 or type 2 with operable concomitant malignancy; type IIIB-type 1 or type 2 with inoperable concomitant malignancy. Our patient had predominantly intra-hepatic lesion with extension to proximal extra-hepatic biliary tree. Pathologically, our patient had type 4 biliary IPMN with type III A cholangiographic pattern.

The clinical manifestations of BT-IPMN are varying and consist of upper abdominal discomfort, biliary colic, intermittent fever and jaundice. These symptoms are usually due to the recurrent obstruction of the bile ducts with cholangitis. [10] The diagnosis of the disease is often incidental during evaluation of a patient with abdominal symptoms. Imaging modalities used for the study of BT-IPMN are same as that for evaluation of any biliary pathology; USG, computed tomogram, MRI and endoscopic retrograde cholangiopancreatography (ERCP). [10] The use of ERCP is helpful for a pre-operative diagnosis as it can demonstrate the presence of mucin draining from the bile duct and it can detect the presence and the location of the suspected neoplasm in biliary tree. Moreover, ERCP permits biopsy confirmation of the disease. [6],[10] The ideal treatment strategy for the disease is yet to be standardized. But higher resectability rate, rare lymph node involvement, late metastasis, and relatively longer survival rates of IPMN-BT, demands a more aggressive surgical approach in treatment. [4] The prognosis of the malignant BT-IPMN is generally thought to be better when compared to other types of cholangiocarcinoma. [4],[6]

 
   References Top

1.
Kim HJ, Kim MH, Lee SK, Yoo KS, Park ET, Lim BC, et al. Mucin-hypersecreting bile duct tumor characterized by a striking homology with an intraductal papillary mucinous tumor (IPMT) of the pancreas. Endoscopy 2000;32:389-93.  Back to cited text no. 1
    
2.
Shibahara H, Tamada S, Goto M, Oda K, Nagino M, Nagasaka T, et al. Pathologic features of mucin-producing bile duct tumors: Two histopathologic categories as counterparts of pancreatic intraductal papillary-mucinous neoplasms. Am J Surg Pathol 2004;28:327-38.  Back to cited text no. 2
    
3.
Zen Y, Fujii T, Itatsu K, Nakamura K, Minato H, Kasashima S, et al. Biliary papillary tumors share pathological features with intraductal papillary mucinous neoplasm of the pancreas. Hepatology 2006;44:1333-43.  Back to cited text no. 3
    
4.
Barton JG, Barrett DA, Maricevich MA, Schnelldorfer T, Wood CM, Smyrk TC, et al. Intraductal papillary mucinous neoplasm of the biliary tract: A real disease? HPB (Oxford) 2009;8:684-91.   Back to cited text no. 4
    
5.
Lee SS, Kim MH, Lee SK, Jang SJ, Song MH, Kim KP, et al. Clinicopathologic review of 58 patients with biliary papillomatosis. Cancer 2004;100:783-93.  Back to cited text no. 5
    
6.
Yeh TS, Tseng JH, Chiu CT, Liu NJ, Chen TC, Jan YY, et al. Cholangiographic spectrum of intraductal papillary mucinous neoplasm of the bile ducts. Ann Surg 2006;244:248-53.  Back to cited text no. 6
    
7.
Lim JH, Jang KT, Choi D. Biliary Intraductal Papillary-Mucinous Neoplasm Manifesting Only as Dilatation of the Hepatic Lobar or Segmental Bile Ducts: Imaging Features in Six Patients. AJR Am J Roentgenol 2008;191:778-82.  Back to cited text no. 7
    
8.
Hruban RH, Takaori K, Klimstra DS, Adsay NV, Albores-Saavedra J, Biankin AV, et al. An illustrated consensus on the classification of pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasms. Am J Surg Pathol 2004;28:977-87.  Back to cited text no. 8
    
9.
Chen TC, Nakanuma Y, Zen Y,Chen MF, Jan YY, Yeh TS, et al. Intraductal papillary neoplasia of the liver associated with hepatolithiasis. Hepatology 2001;34:651-8.  Back to cited text no. 9
    
10.
Lim JH, Yoon KH, Kim SH. Intraductal papillary mucinous tumor of the bile ducts. Radiographics 2004;24:53-66.  Back to cited text no. 10
    

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Correspondence Address:
Raveendran Subhash
Department of Surgical Gastroenterology, Government Medical College , Thiruvananthapuram, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.142676

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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