A 60-year-old man visited his physician because of a skin discoloration suggestive of jaundice, dark urine and pale stools. The patient also reported vague epigastric pain, with onset about 6 months prior to the onset of icterus. He denied any fever or weight loss. His medical history also included hypertension, diabetes mellitus and tuberculosis. A complete blood cell count was taken, which revealed no abnormality, while the blood chemistry profile demonstrated direct hyperbilirubinemia (total bilirubin, TBIL:20 mg/dl, direct bilirubin, DBIL:16 mg/dl) and a remarkable elevation of the alkaline phosphatase (ALP) and gamma-glutamyl transpeptidase (GGT) (the levels of aspartate transaminase (AST) and alanine transaminase (ALT) were only mildly elevated). The patient was subjected to ultrasound examination of his abdomen which disclosed a cystic mass in the head of the pancreas and a dilatation of the common bile duct (1.7 cm) and intrahepatic bile ducts.
The patient was further evaluated with T1-weighed and T2-weighed gadolinium ((Gd)-DTPA)-enhanced MRI images and MRCP, which revealed a cystic lesion in the pancreatic head with maximum transverse diameter of 5 cm. The pancreatic cyst was in communication with a clearly dilated main pancreatic duct. In parallel with the dilatation of the main pancreatic duct along its entire course, a significant dilatation of secondary ducts (side-branches) was also documented (Figure 1). The imaging findings were compatible with the diagnosis of a diffusely distributed intraductal papillary mucinous neoplasm (IPMN) of the mixed-type variety. Moreover, the ultrasonographic finding of the dilated intra- and extra-hepatic biliary tree was confirmed, with maximum diameter of the common bile duct at about 1,7 cm. Due to the level of icterus and the coexisting dilation of the common bile duct (CBD), the patient was subsequently subjected to ERCP with simultaneous insertion of a plastic stent into the CBD. During the upper gastrointestinal endoscopy, only a mild esophagitis of the lower third of the esophagus was diagnosed, with no indication of a gastric wall abnormality reported.
After the aforementioned complete work-up of the patient, he was referred to our Surgical Department for surgical treatment. Because of the diffuse distribution of the cystic neoplasm, a total pancreatectomy, splenectomy and limited partial gastrectomy was performed. Incidentally a subserosal polypoid tumor was found at the greater curvature of the gastric antrum. Local excision of the gastric tumor was performed and it was also sent for histologic examination. On the 6th postoperative day, the patient presented a biliary leak which was managed conservatively. He was discharged on the 40th day, and 1 year after the operation has been disease and symptom free.
Histologic examination of the orthotopic pancreas revealed a non-invasive intraductal papillary mucinous neoplasm involving the main pancreatic duct, with prominent intraductal papillary projections (Figure 2a). The papillae were well-developed with a fibrovascular core. The neoplastic epithelial cells showed intestinal differentiation. The neoplasm exhibited significant architectural and nuclear atypia. There was budding off of clusters of neoplastic cells into the lumen, as well as, significant nuclear pleomorphism with loss of polarity and prominent nucleoli (IPMN with high grade dysplasia; figure 2b).
Additionally, a 2.5 x 2.2 x 0.9 cm measuring tissue specimen from the gastric wall was received. Macroscopic evaluation revealed a 1.5 cm white nodule with cystic spaces. Histological examination demonstrated heterotopic pancreatic tissue consisting of well-formed lobules of pancreatic acini and cystically dilated ducts containing intraluminal papillae (Figure 3a, b). The papillary structures were lined by mucinous epithelium with focal intestinal metaplasia and mild to moderate nuclear atypia (Figure 3b, c).