CASE PRESENTATION
A 52-year-old woman with no prior relevant medical history or chronic medication was referred to outpatient Gastroenterology (GE) consultation after going to the emergency department (ED) for recurrent abdominal pain. In the previous five months, she noticed postprandial epigastric discomfort radiating to her back, nausea and bloating; she also linked the pain’s escalation to stress and certain foods such as vegetables and fruit. In recent weeks, the pain has become more frequent and acute. The system review was otherwise unremarkable.
Observation reveals a soft, non-tender, and modestly swollen abdomen on physical examination, with no palpable lumps or audible bowel sounds. With the exception of gamma-glutamyltransferase (GGT) in the upper range of normal – 40U/L (34), laboratory tests were unremarkable. The results of the other liver tests, amylase and lipase, were normal. An emergency room CT scan revealed a heterogeneous 3cm mass of uncinate pancreatic process with calcifications; the pancreatic duct was not dilated (figure 1). She was prescribed simethicone and put on a FODMAPs diet before being referred to a GE consultation for further evaluation.
She experienced some symptom relief, with decreased stomach bloating and distention but the abdominal pain did not improve significantly. An endoscopic investigation, abdominal MRI and EUS were requested. Endoscopy of the upper gastrointestinal tract and colonoscopy were unremarkable. EUS revealed a 2.7 cm mixed well-delimited pancreatic uncinate mass with no e-flow (figure 2). The biliary and pancreatic ducts were not dilated. No swollen lymph nodes were found. Fine needle aspiration (FNA) was performed (22Gx1, from the second duodenal region, xanthochromic fluid). Endoscopic findings pointed to a neuroendocrine tumor with cystic degeneration. The smears were sparsely cellular with isolated cells positive for CKAE1/AE3 (dot-like) and CD56, and negative for chromogranin. Although it was insufficient for a conclusive diagnosis, the features suggested a neuroendocrine neoplasia/hyperplasia.
A 2.5cm well-circumscribed ovalated cystic mass with some septs was discovered in the uncinated process of the pancreas on abdominal MRI, with an unclear etiology (figure 3). Serum chromogranin was within normal limits. Ga-68 DOTANOC PET was ordinary. The case was discussed in Hepatobiliary and Pancreatic multidisciplinary meeting and surgical resection (pancreatoduodenectomy) was proposed.
The patient had a pancreatoduodenectomy and recovered well afterward. Histologicaly, it was a neoplasm with a solid component and pseudopapillary structures (figure 4). The cells expressed CD10, CD56, PR and B-catenin but were negative for CAM5.2, chromogranine and trypsin. These findings allowed a diagnosis of SPN.  The patient was placed on a surveillance regimen and no additional treatment was given. She is currently asymptomatic six months after surgery.