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A Rare Diagnosis of Intraductal Tubulopapillary Neoplasm of the Pancreas after Pancreaticoduodenectomy

Intraductal tubulopapillary neoplasm (ITPN) of the pancreas is a rare, premalignant tumor characterized by intraductal tubulopapillary growth, ductal differentiation, high-grade cellular dysplasia, and the presence of little or no mucin production.14 In 2010, the World Health Organization classified ITPN as a subclass of intraductal neoplasms of the pancreas, distinct from intraductal papillary mucinous neoplasm.14 ITPNs of the pancreas account for less than 1 per cent of all exocrine pancreatic neoplasms and approximately 3 per cent of all intraductal pancreatic neoplasms.1, 2, 4 Roughly two-thirds of patients can have a clinical presentation that includes nonspecific abdominal pain, vomiting, fever, diabetes exacerbation, and/or weight loss.4 However, up to one-third of patients are asymptomatic with incidental discovery of the lesion.13 Because of the rarity of ITPN, there are few case reports available, and information regarding the disease is limited. In this case, we review the presentation of ITPN diagnosed in an elderly female with abdominal pain and elevated liver function tests. A 74-year-old female with past medical history of nonmelanoma skin cancer, paroxysmal atrial fibrillation, heart disease, hyperlipidemia, thyroid disease, arthritis, and asthma was found to have mildly elevated transaminases on routine laboratory studies. Upon further evaluation, she was found to have a six month history of episodic epigastric abdominal pain that subsided without intervention. The patient admitted to a history of weight loss and poor appetite. She denied any history of nausea or vomiting and had no previous history of pancreatitis. Physical examination revealed epigastric abdominal fullness, right upper quadrant, and epigastric tenderness. The patient underwent evaluation with a CT, which identified a 5.7-cm solid and cystic mass involving the posterior aspect of the pancreatic head. Additional evaluation with MRI revealed mass effect to the distal aspect of the common bile duct and to the main pancreatic duct with secondary pancreatic and intrahepatic biliary dilatation, without clear invasion of these structures (Figs. 1 and 2). In addition, there was secondary dilatation of the gallbladder and atrophy of the pancreatic parenchyma. The pancreatic head mass was further evaluated with endoscopic ultrasound. Findings confirmed the mass to be highly vascular, with both solid and cystic features. The degree of vascular flow throughout the lesion raised concern for possible bleeding complications and prevented endoscopic biopsy. The patient was not jaundiced on examination and LFTs including total bilirubin was normal preoperatively. However, the carcinoembryonic antigen and CA 19-9 levels were elevated at 12.3 ng/mL (normal range: <5.0 ng/mL) and 39 U/mL (normal range: <37 U/mL), respectively. An open pancreaticoduodenectomy using a right subcostal incision was performed, along with the placement of a gastrojejunostomy tube, without complications. Macroscopic examination of the Whipple specimen revealed a 5.5-cm solid and cystic tumor. The cystic portion comprised 25 per cent of the mass and contained clear, serous fluid. The tumor was encapsulated with no gross evidence of invasion. Histological examination of the Whipple specimen revealed high-grade dysplasia and carcinoma in situ with negative margins. The intraductal tumor nodules were tubular and solid. The papillary configuration within the tumor was more prominent than what is usually seen in ITPN. There was no evidence of mucin production within the tumor specimen. Regional lymph nodes did not exhibit signs of metastasis. Immunohistochemical staining was positive for CDX2 expression, which indicates focal intestinal differentiation but Presented at the Southeastern Surgical Congress, Conference, February 12, 2018, Tampa, FL. Address correspondence and reprint requests to Allison Cauthen, B.S., Mercer University School of Medicine, 1501 Mercer University Drive, Macon, GA 31207. E-mail: abcauthen11@gmail. com.