A 77-year-old Caucasian woman was admitted to Panevezys regional hospital in Lithuania because of a 1-day history of skin and scleral jaundice and weakness. Her medical history included a laparoscopic cholecystectomy 15 years earlier for a gallbladder stone.
A clinical examination revealed only skin and scleral jaundice. The patient's laboratory test results were as follows: total bilirubin (TBIL) 338.59 U/L (normal <21 U/L), direct (conjugated) bilirubin (DBIL) 294 U/L (normal <5.3), alkaline phosphatase (ALP) 720.1 U/L (normal 40-129), alanine transaminase (ALT) 52.0 U/L (<=35), aspartate transaminase (AST) 142.1 U/L (normal <=35), gamma-glutamyl transpeptidase (GGT) 353.0 U/L (normal 9-40), and carbohydrate antigen 19-9 157.09 U/ml (normal 0-37). On the basis of ultrasound and computed tomography (CT) findings, cancer in the head of the pancreas with invasion to the common bile duct (CBD) was suspected. To alleviate jaundice and to perform brush cytology, percutaneous transhepatic bile duct drainage was performed. After this procedure, decreases in TBIL (187.92 U/L), DBIL (95.69 U/L), ALP (276.1 U/L), ALT (37.2 U/L), AST (73.0 U/L), and GGT (123.8 U/L) were noted.
A bile duct villous papilloma was suspected on the basis of brush cytology. Despite the results of these investigations, a chance of malignancy remained. The patient was referred to the local tertiary treatment center for further treatment. At the Vilnius University Hospital Santariskiu Klinikos, a multidisciplinary team reviewed a CT scan of the patient. The scan showed the presence of a CBD-obstructing, homogeneous, 5 x 3-cm mass below the fusion of extrahepatic ducts without any evidence of tumor metastasis in a common bile duct surrounding tissue, the liver, right and left intrahepatic ducts, and the pancreas. The patient's periportal lymph nodes were of normal size. On the basis of the tumor's radiological features, a preoperative diagnosis of extrahepatic bile duct malignant tumor was made (T1N0M0, type I, by Bismuth-Corlette classification) (Fig. 1).
The multidisciplinary team assessed the absence of tumor invasion in surrounding tissues and decided to perform a surgical resection of the tumor. However, there remained a possibility to perform a more radical operation, such as pancreaticoduodenectomy, if the cancer was observed to involve the distal part of the CBD.
A laparotomy was performed. The duodenum and the head of the pancreas were mobilized by using the Kocher maneuver. Importantly, no trace of tumor was found in the proximal common hepatic duct (CHD) and distal CBD parts. Only bile duct parts containing tumor were resected. To perform a Roux-en-Y hepaticojejunostomy, a small intestine loop was mobilized and divided 50 cm from the superior duodenal fold. The distal part was closed and anastomosed end to side to the remaining 0.5-cm length of the CHD. Sixty centimeters below this junction, an end-to-side jejunojejunal anastomosis was formed.
Macroscopic evaluation of the specimen revealed that the resected parts of the CBD and CHD consisted of dilated bile ducts with a papillary tumor protruding into the lumen. The tumor's size was 4.5 x 4 x 2.5 cm. The resection margin was 2 cm from one side and 2.5 cm from the other, and two investigated lymph nodes were identified as tumor-free (Fig. 2).
Histologically, the tumor consisted of branching papillary structures lined by tall columnar epithelium with slight nuclear atypia and scant fibrous stroma (Fig. 3a, b). Invasive growth was not detected. The adenoma was tested for microsatellite instability using immunostaining of MLH1, PMS2, MSH2, and MSH6 proteins. Positive nuclear immunostaining was observed in tumor cells (Fig. 3c), which showed no evidence of microsatellite instability.
The patient's postoperative course was uneventful. Her hospitalization period was 10 days. At two visits during the 18-month follow-up period, she had no complications or recurrence.