Hypervascular pancreatic mass with multiple peptic ulcers and thickened folds
• Best imaging tool
Helical CT or MR for pancreas and possible metastasis
Endoscopic ultrasonography for additional primary sites; guides biopsy
TOP DIFFERENTIAL DIAGNOSES
• Helicobacter pylori gastritis
• Gastric carcinoma
• Gastric metastases and lymphoma
• Extrinsic inflammation
• Other gastritides
PATHOLOGY
• 20-60% of cases are associated with multiple endocrine neoplasia type 1 (MEN1)
CLINICAL ISSUES
• Most common signs/symptoms
Pain, increased acidity, severe reflux, diarrhea, upper gastrointestinal tract ulcers
Gastrinomas are often multiple (60%), malignant (60%), and metastatic (30-50%)
• Hypergastrinemia is hallmark of Zollinger-Ellison syndrome (ZES)
Serum gastrin level of > 1,000 pg/mL is virtually diagnostic of ZES
• Prognosis
Good with surgical resection of primary gastrinoma
Poor if gastrinoma, liver metastases, or ulcers recur after surgery
(Left) Axial CECT in a 63-year-old man who presented with intractable peptic ulcer disease demonstrates hyperemia and mural thickening of the stomach.
(Right) Arterial phase CECT in the same patient shows a small hypervascular gastrinoma in the pancreatic head. It is important to distinguish this from the superior mesenteric artery and superior mesenteric vein .
(Left) Axial CT of a 55-year-old woman with hypercalcemia, diarrhea, and severe abdominal pain as presenting symptoms of MEN1 syndrome shows one of several neck masses , representing parathyroid adenomas or hyperplasia.
(Right) Abdominal CT in the same case shows marked hypervascularity and thickening of the gastric wall . Multiple liver metastases are present . The serum gastrin levels were strikingly elevated, confirming ZES, though the gastrinoma was not identified on CT.
TERMINOLOGY
Abbreviations
• Zollinger-Ellison syndrome (ZES)
Definitions
• Severe peptic ulcer disease associated with marked ↑ in gastric acid due to gastrin-producing endocrine tumor (gastrinoma) of pancreas
IMAGING
General Features
• Best diagnostic clue
Hypervascular pancreatic mass with multiple peptic ulcers and thickened folds
• Location
Gastrinoma: Pancreas (75%), duodenum (15%), and liver and ovaries (10%)
– Common site: Gastrinoma triangle
Superiorly: Cystic and common bile ducts
Inferiorly: 2nd and 3rd parts of duodenum
Medially: Junction of pancreatic neck and body
Ulcers: Stomach and duodenal bulb (75%), postbulbar and jejunum (25%)
Radiographic Findings
• Barium studies: Gastric, duodenal, and proximal jejunum
Large volume of fluid dilutes barium and compromises mucosal coating
Markedly thickened gastric folds
Peptic ulcers: Round or ovoid barium collections surrounded by thin or thick radiolucent rim (edematous mucosa) and radiating folds
CT Findings
• Gastrinomas
Small or large, heterogeneous density lesion, ± cystic and necrotic areas, ± calcification
Liver metastases are common
Hypervascular (primary and secondary) lesions ± local or vascular invasion on arterial and portal venous phase
Inflammatory changes in stomach, duodenum, and proximal small bowel
– Thickened gastric, duodenal, and jejunal folds
Signs of ulcer penetration
– Wall thickening, luminal narrowing of stomach and duodenum
Signs of ulcer perforation
– Free air in abdomen (from a duodenal or antral ulcer) or lesser sac (from a gastric ulcer)
MR Findings
• T1WI
Hypointense pancreatic nodule on fat-saturated sequence
• T2WI
Hyperintense on spin-echo sequence
– Both primary and metastatic tumors
• T1WI C+
Hyperintense, hypervascular on fat-saturated delayed spin-echo sequence
Ultrasonographic Findings
• Endoscopic ultrasonography (EUS)
Detects small gastrinomas better than CT or MR
Usually homogeneously hypoechoic mass
• Intraoperative ultrasonography
Detects very small tumors (75-100% sensitivity)
Angiographic Findings
• Conventional
Hypervascular gastrinomas and metastases
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of the stomach.
in the pancreatic head. It is important to distinguish this from the superior mesenteric artery 
and superior mesenteric vein 
.
, representing parathyroid adenomas or hyperplasia.
. Multiple liver metastases are present 
. The serum gastrin levels were strikingly elevated, confirming ZES, though the gastrinoma was not identified on CT.
