title: The clinicopathological spectrum and management of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B).
abstract: BACKGROUND
  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.
  
  
  METHODS
  Clinical, radiological and histopathological data of seven consecutive patients who were found to have IPMN-B were analyzed.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Clinical Science, Intervention, and Technology (CLINTEC), Division of Surgery, Karolinska Institutet, Karolinska University Hospital, Huddinge, Stockholm, Sweden. melroy.dsouza@karolinska.se
authors: Melroy A
authors: MA
authors: D'souza
authors: Bengt
authors: B
authors: Isaksson
authors: Matthias
authors: M
authors: Löhr
authors: Lars
authors: L
authors: Enochsson
authors: Fredrik
authors: F
authors: Swahn
authors: Lars
authors: L
authors: Lundell
authors: Urban
authors: U
authors: Arnelo
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D000231:Adenocarcinoma, Papillary / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D000368:Aged; D001650:Bile Duct Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D002760:Cholangiopancreatography, Endoscopic Retrograde; D002761:Cholangitis / Q000209:etiology; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy / Q000379:methods; D006801:Humans; D041781:Jaundice, Obstructive / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D010180:Pancreatectomy; D016896:Treatment Outcome