Mucin-producing bile duct tumor treated successfully with endoscopic ultrasound-guided hepaticogastrostomy.

We report the case of an 82 year-old Japanese man with a history of multiple heart surgeries who presented with nausea and loss of appetite. Laboratory tests showed elevated liver and biliary enzymes. Imaging showed a possible space-occupying lesion within a dilated bile duct. Endoscopic retrograde cholangiography showed an intact ampulla of Vater with a visible mucus-filled orifice presenting a fish-mouth appearance and fluoroscopy showed a possible tumor-causing mild stenosis of the bile duct. Cholangioscopy revealed papillary protrusions in the common bile duct. Pathological evaluation was inconclusive but negative for malignancy. Patient was presumptively diagnosed with intraductal papillary neoplasm of the bile duct. Mucobilia caused repeated migration of metallic biliary stents, requiring numerous endoscopic interventions. Endoscopic ultrasound-guided hepaticogastrostomy was performed, as patient was no longer fit for surgery. All abdominal symptoms resolved and laboratory values normalized. Patient remained symptom-free during 12 months of follow-up.

Mucobilia: An Unusual Cause of Persistent Jaundice.

Mucobilia is defined as the abnormal secretion of copious amounts of mucus from within the bile ducts, manifesting as obstructive jaundice or cholangitis. A spectrum of mucin-producing hepatobiliary and pancreatic neoplasms have been associated with mucobilia. We report the case of a 63-year-old male with an intestinal type of intraductal papillary neoplasm of the intrahepatic bile duct.

Recurrent Acute Pancreatitis Caused by Mucin-Producing Liver Metastases.

We present an atypical case of a 69-year-old woman with recurrent episodes of acute pancreatitis and a background history of mucinous colorectal adenocarcinoma. It is emphasized that in the framework of biliary malignant obstruction, modern non-invasive techniques like Magnetic Resonance Imaging (MRI) with hepatospecific contrast agent and Positron Emission Tomography (PET) may help establish the specific cause of the obstruction before any biliary decompression procedure is carried out. This strategy has the potential to prevent from more important tumour spread and provide a greater chance for curative surgery when applicable.

Clinicopathological features and prognosis of mucin-producing bile duct tumor and mucinous cystic tumor of the liver: a multi-institutional study by the Japan Biliary Association.

BACKGROUND: The aim of this study was to determine the clinicopathological features and surgical outcomes of mucinous cystic neoplasm of the liver (MCN) and mucin-producing intraductal papillary neoplasm of the intrahepatic bile duct (M-IPNB). METHODS: We performed a multi-institutional, retrospective study of patients with MCN or M-IPNB pathologically defined by the presence or absence of an ovarian-like stroma. RESULTS: The M-IPNB and MCN were diagnosed in 119 and nine patients, respectively. MCN was observed in female patients, while M-IPNB produced symptoms of cholangitis. M-IPNBs were classed as low or intermediate grade in 53 cases, high grade in 23 and invasive carcinoma in 43. Fifty-one of the M-IPNBs were the pancreatobiliary type (PT), 33 were the intestinal type (IT), 23 were the oncocytic type (OT), and 12 were the gastric type (GT). The 1-, 5- and 10-year survival rates for the 105 patients with M-IPNB were 96%, 84% and 81%, respectively, while the 5-year survival rate for patients with MCN was 100%. OT and GT M-IPNB had better 10-year survival rates than PT and IT M-IPNB. CONCLUSIONS: Although MCN has different features from M-IPNB, both diseases have a good prognosis after resection. The cellular type of M-IPNB appears to predict outcome.

A case of intraductal papillary neoplasm of the bile duct suspected to be of peribiliary glands origin.

Peribiliary glands are complex lobular structures containing mucus and serous glands, distributed along the extrahepatic and intrahepatic bile ducts. In this report, we describe a case of intraductal papillary neoplasm of the bile duct suspected to be of peribiliary glands origin. The patient was an 80-year-old man who was referred to our hospital for a hepatic mass. On further examination, a 38 × 34 mm cystic lesion with papillary growth was found in S1/4. Because the lesion was extensively bordered by both hepatic ducts and the connection was unclear, it was difficult to determine the extent of hepatic resection. To confirm the location, a peroral cholangioscopy was performed. The connection with the cyst was detected in the right hepatic duct and a villous tumor mucosa protruded through the conduit lumen. Since we found that the lesion communicated with the right hepatic duct, a right hepatectomy was subsequently performed. The postoperative pathological diagnosis was an intraductal papillary neoplasm of the blie duct with associated invasive carcinoma. The postoperative course was good, and the patient experienced no recurrence.