J Gastrointest Surg (2015) 19:792–794 DOI 10.1007/s11605-015-2750-2

GI IMAGE

Intraductal Papillary Mucinous Neoplasm of the Liver: GI Image Andrew Mo & Gabriel Brat & Gaya Spolverato & Timothy M. Pawlik

Received: 15 December 2014 / Accepted: 8 January 2015 / Published online: 24 January 2015 # 2015 The Society for Surgery of the Alimentary Tract

Abstract Intraductal papillary mucinous neoplasms (IPMNs) are rare, mucin-producing, predominantly noninvasive tumors arising from epithelial cells. Most IPMNs arise from the pancreas. There exists a subset of IPMN of the biliary tract (BT-IPMN). IPMNs regardless of origin produce large amounts of mucin relative to scant amounts of epithelial cells, leading to mass effect disturbances in bile flow. Affected bile ducts exhibit marked dilatation. The majority of IPMN patients present with a non-malignant neoplasm. Because of potential for transformation, surgery is the treatment of choice. Bronchobiliary fistulas can present as congenital defects, following thoracoabodominal trauma, or as rare complications of diseases of the biliary tract such as BT-IPMN. There are no reported cases in the literature of bronchobiliary fistula associated with BT-IPMN, but there is a clear theoretical risk. Keywords IPMN . Bile duct . GI image

Clinical Data A 66-year-old Caucasian man with a history of Crohn’s disease and primary sclerosing cholangitis presented with 6 months of progressive weight loss, fatigue, poor taste in his mouth, productive mucinous cough, and scleral icterus. He also reported diarrhea, pale stools, and dark urine. Biliary sclerosis had led to common bile duct resection with hepaticojejunostomy 3 years ago. Pathology reports from the patient’s previous resection showed no evidence of malignancy. One year later, he experienced recurrent jaundice. At that time, percutaneous transhepatic cholangiography was performed followed by stent placement for a stricture of the hepaticojejunostomy. At presentation, the output from the transhepatic biliary drains was mucinous. Laboratory results indicated mild anemia (hemoglobin, 10.9 g/dL), elevated transaminase levels (aspartate aminotransferase/alanine aminotransferase, 93/114 U/L), bilirubin level of 13.6 mg/dL, and alkaline phosphatase of 169 U/L. Computed tomographic images from an outside institution were reviewed in-house and compared to existing records. There was an increased size of a previously noted cystic mass in the right liver (Fig. 1a) with invasion A. Mo : G. Brat : G. Spolverato : T. M. Pawlik (*) Department of Surgery, Johns Hopkins Hospital, 600 N. Wolfe Street, Blalock 688, Baltimore, MD 21287, USA e-mail: [email protected]

through the diaphragm into the right lower lobe and through the chest wall. Associated narrowing of the right portal vein was present (Fig. 1b, yellow arrow). Increasing retroperitoneal and upper abdominal adenopathy and fullness of the pancreatic head were noted. The patient was taken to the operating room for excision of the hepatic lesion, repair of the diaphragm, and diagnosis. A mucinous hepatic neoplasm (invasive intraductal papillary mucinous neoplasms) was discovered intraoperatively at the location of the previously noted lesion (Fig. 2). The mucinous neoplasm was found to have fistulized through the diaphragm with formation of a bronchobiliary fistula. A non-anatomical hepatectomy of the right liver was performed along with partial wedge resection of the middle and lower lobes of the lung. A segment of diaphragm was resected and primarily repaired. Pathology of surgical specimens showed invasive, moderately differentiated mucinous adenocarcinoma arising in an intraductal papillary mucinous neoplasms (IPMN) of the liver. The dimensions of the lesion were 13.0 cm×7.0 cm×5.5 cm with involvement of the medial margin.

Discussion IPMNs are rare, mucin producing, predominantly noninvasive tumors arising from epithelial cells. Most IPMNs arise from the pancreas. There exists a subset of IPMN of the biliary tract (BT-IPMN).1 These neoplasms are the biliary counterpart of the pancreatic IPMN.2 Differences include higher serum levels of CEA and CA19-9 and higher pathological diagnosis of malignancy in BT-IPMNs compared to

J Gastrointest Surg (2015) 19:792–794

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possible. Severe dilation of bile ducts may be the only radiographic finding. In these cases, a definitive diagnosis of BTIPMN can be made only after resection.5 The majority of IPMN patients present with a benign, non-invasive neoplasm. Because of potential for transformation, surgery is the treatment of choice. Depending on the location, this may include bile duct resection, pancreatoduodenectomy, and hepatic resection. Long-term survival is expected for benign, noninvasive IPMNs.6 In rare cases, an invasive carcinoma is , present.2 6 Surgery remains the best option for both invasive and non-invasive IPMNs, albeit with worse survival outcomes in invasive IPMNs. Bronchobiliary fistulas are an abnormal communication between the biliary channels and the bronchial tree. Biloptysis, the presence of bile in sputum, is pathognomic.7 Bronchobiliary fistulas can present as congenital defects, following thoracoabodominal trauma, or as rare complications of diseases of the biliary tract such as BT-IPMN. There are no reported cases in the literature of bronchobiliary fistula associated with BT-IPMN, but there is a clear theoretical risk. Bile is a strong irritant and can cause inflammatory reactions in the subdiaphragmatic space when present secondary to trauma or disease, eroding the diaphragm and creating a passage between the biliary tract and bronchial tree.8

Conclusion Fig. 1 a Cystic hepatic mass. b Narrowing of right portal vein (yellow arrow)

IPMNs of the pancreas.1 IPMNs, regardless of origin, produce large amounts of mucin relative to scant amounts of epithelial cells, leading to mass effect disturbances in bile flow. Affected bile ducts exhibit marked dilatation.3 Tumors are confined within the bile ducts and rarely invade surrounding margins.4 On imaging, visualization of the neoplasm may not be

Bronchobiliary fistula can be a pattern of presentation for BTIPMN, thus an interdisciplinary approach to patients with complications of diseases of the biliary tract, such as BTIPMN, is crucial.

IRB Approval Done Conflict of Interest None

References

Fig. 2 Cystic hepatic lesion

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