Biliary mucinous cystic neoplasm (BMCN) is a rare intrahepatic neoplasm comprising approximately 5% of cystic liver lesions. It can cause diagnostic dilemmas with most common differentials being complex hepatic cyst, hydatid cyst and intraductal papillary neoplasm of the bile duct. Affecting middle-aged female population, BMCN presents variedly ranging from vague abdominal symptoms to obstructive jaundice. Preoperative diagnosis is difficult. Preoperative CT scan with intravenous contrast and carbohydrate antigen 19.9 levels may give a clue towards the diagnosis. Intraoperative frozen section or cyst fluid aspiration cytology might help confirm the diagnosis. Fine needle aspirations should not be performed if BMCN is suspected, and intraoperative cyst spillage should be avoided to prevent tumour dissemination. We present here a case with a very atypical presentation of BMCN and review its present literature in brief.

A 47-year-old male with history of alcohol abuse for 8 to 10 years visited the Department of Gastrointestinal Surgery for recurrent attacks of mild acute pancreatitis since 3 years. He also had recurrent epigastric pain, significant weight loss, early satiety and postprandial fullness. Multiple ultrasonography examinations and Computed Tomography (CT) scans of abdomen done in the past suggested chronic pancreatitis for which patient was advised pancreatic enzyme supplementation, alcohol abstinence, analgesics and dietary changes by different physicians, only to recur again.CT scan revealed bulky head and uncinate process of pancreas with a hypodense area between pancreatic head and second part of duodenum [Table/ Fig-1], prominent pancreatic duct, dilated Common Bile Duct (CBD) (15mm), hugely distended gall bladder, irregular wall thickening of first and second part of duodenum with stenosis of second part of duodenum [Table/ Fig-2]. Upper gastrointestinal endoscopy did not reveal significant duodenal obstruction. Duodenal biopsy showed Brunner's gland hyperplasia. Endoscopic Ultrasound (EUS) confirmed the CT findings. An Fine Needle Aspiration Cytology (FNAC) showed only inflammatory infiltrate and no malignancy. Blood level of CA19-9 was normal. Liver function tests were normal.A diagnosis of groove pancreatitis was made. In view of inability to exclude a neoplasm with certainty, the patient was subjected to a pylorus preserving Pancreatoduodenectomy (PD). During surgery a hard mass in the head and the uncinate process of pancreas was felt. There was no pancreatic calcification. A dilated CBD and a fibrotic partially constricting ring in the midportion of second part of the duodenum were noted.Histopathological examination [Table/ Fig-3] revealed islands of heterotopic pancreatic tissue in the muscularis propria of the duodenum. The lobules comprised of unremarkable pancreatic acini, ducts and few clusters of islet cells. The acini were atrophic in places and few aggregates of ductules were seen. Cystic dilatation of ductules was seen in the duodenal wall with mild to moderate inflammatory infiltrate. Extensive brunner gland hyperplasia was seen in mucosa and submucosa of the duodenum leading to widening of submucosa. The duodenal muscle was markedly hypertrophied, especially in its medial wall, which contained the heterotopic pancreatic tissue. Both these features probably led to narrowing of the duodenal lumen. The head of pancreas showed focal evidence of chronic pancreatitis. The postoperative course was uneventful. The patient is asymptomatic five years after the surgery. A diagnosis of groove pancreatitis was made. The patient was subjected to a pylorus preserving PD. Histopathological examination showed cystic dystrophy of duodenum due to heterotopic pancreas. The patient is doing well at 5 years of follow-up.[

Background: Majority of patients with large size HCC (>10 cm) are not offered surgery as per Barcelona Clinic Liver Cancer (BCLC) criteria and hence, their outcomes are not well studied, especially from India, owing to a lower incidence. Aim: To analyze outcomes of surgery for large HCCs. Methods: This retrospective observational study included all patients who underwent surgery for large HCC from January 2007 to December 2017. The entire perioperative and follow up data was collected and analyzed. Results: Nineteen patients were included. Ten were non-cirrhotic; 16 were BCLC grade A; one BCLC grade B; and two were BCLC C. Two cirrhotic and three non-cirrhotic underwent preoperative sequential trans-arterial chemoembolization and portal vein embolization. Right hepatectomy was the most commonly done procedure. The postoperative 30-day mortality rate was 5% (1/19). Wound infection and postoperative ascites was seen in seven patients each. Postoperative liver failure was seen in five. Two cirrhotic and two non-cirrhotic patients had postoperative bile leak. The hospital stay was 11.9±5.4 days (median 12 days). Vascular invasion was present in four cirrhotic and five non-cirrhotic patients. The median follow-up was 32 months. Five patients died in the follow-up period. Seven had recurrence and median recurrence free survival was 18 months. The cumulative recurrence free survival was 88% and 54%, whereas the cumulative overall survival was 94% and 73% at one and three years respectively. Both were better in non-cirrhotic; however, the difference was not statistically significant. The recurrence free survival was better in patients without vascular invasion and the difference was statistically significant (p=0.011). Conclusion: Large HCC is not a contraindication for surgery. Vascular invasion if present, adversely affects survival. Proper case selection can provide the most favorable survival with minimal morbidity.

Highlights Vascular assessment is important in all complex biliary injury cases. Perihepatic/peribiliary collaterals provide adequate blood supply to bile ducts. Balloon dilatation is helpful in biliary-enteric anastomotic strictures. Delayed biliary enteric repair is better in proper hepatic artery block cases. Minimum hilar dissection should be done during definitive repair.

Multiple cholecystoenteric fistulae, Bouveret syndrome (a form of gallstone ileus), and acute pancreatitis occurring together is very rare. Diagnosis is seldom clinical and is mostly based on computerised tomography (CT) or magnetic resonance imaging (MRI). Endoscopy and minimally invasive surgery have revolutionised the treatment of Bouveret syndrome and cholecystoenteric fistula, respectively, over the last two decades. Laparoscopic repair of cholecystoenteric fistula followed by cholecystectomy is successful on a consistent basis with skilled laparoscopic suturing and advanced laparoscopy. In patients with Bouveret syndrome, when the stone is <4cm and is in the proximal duodenum, it is usually amenable for endoscopic extraction with snares, nets, forceps and lithotripsy. When endoscopy is unavailable or fails, laparoscopic surgery is suitable for these patients. However, stones >4 cm, located in the distal duodenum, multiple fistulae, and associated acute pancreatitis may necessitate open surgery. We present here a case of a 65year-old Indian female with multiple cholecystoenteric fistulae and Bouveret syndrome with acute pancreatitis with a 6.5 cm gallstone diagnosed on CT scan and MRI and treated successfully by open surgery. We also review the current literature on the management of this complex problem.