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A woman in her 60s was referred to our hospital for further examination of a pancreatic cystic and solid lesion detected on abdominal ultrasonography. The patient had no particular symptoms. On physical examination, the abdomen was soft and flat with no tenderness. Laboratory data revealed normal serum levels of carcinoembryonic antigen (2.7 ng/mL, reference range 0–5 ng/mL) and carbohydrate antigen 19–9 (2 U/mL, reference range 0–37 U/mL). Contrast-enhanced CT revealed a cystic lesion 13 cm in diameter with solid nodules inside, showing contrast enhancement ( figure 1). MR cholangiopancreatography revealed a large multilocular cystic mass with papillary solid components inside and the common bile duct was compressed (figure 2). Endoscopy revealed mucus excretion from Vater papilla (figure 3A). Endoscopic ultrasonography revealed a large cystic mass with papillary solid components inside the head of the pancreas (figure 3B). Preoperative diagnosis was made with intraductal papillary mucinous neoplasm (IPMN) showing enhanced mural nodule. As the lesion diffusely involved the gland, total pancreatectomy was performed due to increased risk for malignancy (main duct IPMN, larger than 3 cm diameter, enhanced mural nodule). Gross examination revealed a 15 cm cystic and solid neoplasm entirely replacing the parenchyma, filled with mucin (figure 4). The main pancreatic duct was dilated with prominent papillary projections (figure 5A), and tumour cells displayed abundant eosinophilic glandular cytoplasm (figure 5B). Immunohistochemical staining revealed positivity for MUC5AC (figure 6A), MUC6 (figure 6B) and hepatocyte paraffin-1 (figure 6C). Pathological diagnosis was intraductal oncocytic papillary neoplasms (IOPN) with high-grade dysplasia. After surgery, the patient received treatment with insulin for blood glucose control and digestive enzymes for loss of pancreatic exocrine function. The postoperative course was uneventful. After a 3-year follow-up, the patient had no recurrence and was alive.