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.