The clinicopathological spectrum and management of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B). (April 2013)

• Record Type: Journal Article
• Authors: D'souza, Melroy A; Isaksson, Bengt; Löhr, Matthias; Enochsson, Lars; Swahn, Fredrik; Lundell, Lars; Arnelo, Urban
• Is Part Of: Scandinavian journal of gastroenterology. Volume 48:Number 4(2013)
• Page Start: 473
• Page End: 479
• Publication Date: 2013-04
• Subjects: Gastroenterology -- Periodicals; Digestive organs -- Diseases -- Periodicals; 616.33
• DOI: 10.3109/00365521.2012.722672
• Languages: English
• ISSNs: 0036-5521

Abstract

<abstract> <title>Abstract</title> <p> <bold> <italic>Background. </italic> </bold>Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is a rare but increasingly diagnosed clinical entity. Typical cholangioscopic findings usually include intraductal protruding papillary tumors that secrete mucus. <bold><italic>Methods. </italic></bold>Clinical, radiological and histopathological data of seven consecutive patients who were found to have IPMN-B were analyzed. <bold><italic>Results.</italic></bold> Six of the seven patients presented with obstructive jaundice/cholangitis as the presenting complaint. ERCP and other imaging were equivocal in five of these patients and peroral cholangioscopy (POCS, single-operator cholangioscopy system) was performed. This revealed mucin-producing intraductal tumors with numerous frond-like papillary projections; a macroscopic appearance consistent with IPMN-B. Preoperative biopsy revealed adenoma, with low-grade dysplasia in two patients and high-grade dysplasia in three. Three patients underwent Whipple resection; one underwent total pancreatectomy with left hepatectomy, one patient a pancreas preserving duodenectomy with common bile duct reimplantation and one patient an extended right hepatectomy. These patients were found to have IPMN-B with adenomatous changes with varying grades of dysplasia and even cholangiocarcinoma on final histopathology. One patient first underwent endoscopic papillectomy and on follow-up was found to have cholangiocarcinoma with metastases to the liver. <bold><italic>Conclusion. </italic></bold>POCS can be a key diagnostic investigation in the evaluation of patients with papillary tumors of the bile duct. IPMN-B has a heterogenous pathology and varying grades of dysplasia and even carcinoma may exist in the same patient. Surgical management should be radical and based on tumor extent.</p> </abstract>