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An Incidentally Discovered Abdominal Mass During Physical Examination Q2
Tao Li Department of General Surgery, Qilu Hospital, Shandong University, Jinan, P.R. China
Question: A 66year-old woman was referred to our hospital for an incidentally discovered abdominal mass during routine physical examination. She had no history of abdominal trauma, liver cirrhosis, or chronic hepatitis virus infection previously, nor did she have of abdominal pain, jaundice, or cholangitis. Physical examination revealed mild tenderness of the right upper abdominal quadrant. Blood test results, including complete blood count, liver function tests, and tumor markers of carbohydrate antigen 19-9, alpha fetoprotein, and carcinoembryonic antigen were all within normal ranges. Computed tomography scan demonstrated a heterogeneous cyst-like mass (5 6 cm) with slightly enhanced projections along the walls in the arterial phase (Figure A), and the mass seemed to be communicated with the common bile duct (Figure B, arrow). The patient underwent an exploratory laparotomy and the tumor was resected completely. The patient was followed for 3 years without evidence of recurrence. What is the diagnosis of this tumor? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
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Conflicts of interest The author discloses no conflicts. Funding Supported by the grants from the National Natural Science Foundation of China (Grant No. 81572328) and Major Program of Shandong Provincial Natural Science Foundation (Grant No. ZR2014HZ002). © 2016 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2016.03.048
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Answer to: Image 4: Intraductal Papillary Neoplasm of the Bile Duct
At laparotomy a well-defined cystic mass was found emerging from the right hepatic duct and was communicated with the bile duct. The gallbladder was compressed and pushed aside. The lesion was resected and hepatocholangiojejunostomy was performed. Pathologic examination revealed that the lesion was confined within the right hepatic duct wall without any evidence of invasion into the adjacent liver. The cyst contents were mucoid and hemorrhagic and the cyst wall was lined with soft and friable papillary tumor masses with focal nodular, more solid areas. Histologic examination showed the intraductal papillary growth of neoplastic biliary epithelia with fine fibrovascular cores in the lumen of the biliary tree (Figure C), suggesting the diagnosis of intraductal papillary neoplasm of the bile duct (IPN-B). IPN-B is defined as a cystic lesion that is lined with biliary, mucinous, or oncocytic epithelium in papillary configurations without ovarian-like stroma.1 As a newly recognized entity, IPN-B has been reported previously in the literature under various terms, such as biliary papillomatosis, bile duct papillomatosis, or mucin-hypersecreting cholangiocarcinoma. In 2010, the World Health Organization classification system recommended the nomenclature of “intraductal papillary neoplasms of the bile duct” and regards IPN-B as a biliary counterpart of pancreatic intraductal papillary mucinous neoplasms because of the striking similarities between them.1 IPN-B represents a disease spectrum from benign to malignant following the course of chronic inflammation, dysplasia, and carcinoma in situ to invasive carcinoma.2 Hepatolithiasis, clonorchiasis, and chronic proliferative cholangitis are found frequently in the affected liver with IPN-B and are suggested to be related to the origin of IPN-B. Intraductal neoplasia intermingled with mucin or hepatolithiasis is liable to occlude the bile duct, and thereby cause the common symptom of right hypochondralgia, cholangitis, and obstructive jaundice. However, intraductal papilloma and mucin secretion can be detected in fewer than one-half of patients by conventional radiologic modalities; therefore, cholangioscopy is sometimes needed to confirm the histology and extent of the lesions to ensure that appropriate treatment is provided. Currently, IPN-B is considered to be a premalignant disease with different subtypes and malignant potential. Although tumor cell type of IPN-B might affect the growth type of tumors and influence the outcome of the patients, IPN-B should be treated aggressively and resection provides the best chance for cure. An excellent prognosis with long term survival can be expected after complete resection of the tumor.3 If major surgery is not indicated, adjuvant or palliative procedures such as intraluminal radiation and biliary drainage are recommended to prolong survival.
References 1. 2. 3.
Nakanuma Y, Curado MP, Franceschi S, et al. Intrahepatic cholangiocarcinoma. In: Bosman FT, Carneiro F, Hruban RH, et al., eds. WHO classification of tumors of the digestive system. 4th ed. Lyon: IARC Press, 2010:217–224. Yeh TS, Tseng JH, Chiu CT, et al. Cholangiographic spectrum of intraductal papillary mucinous neoplasm of the bile ducts. Ann Surg 2006;244:248–253. Li T, Ji Y, Zhi XT, et al. A comparison of hepatic mucinous cystic neoplasms with biliary intraductal papillary neoplasms. Clin Gastroenterol Hepatol 2009;7:586–593.
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