Zollinger-Ellison Syndrome

Published on 19/07/2015 by admin

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Last modified 22/04/2025

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 Hypervascular pancreatic mass with multiple peptic ulcers and thickened folds

• Best imaging tool

image Helical CT or MR for pancreas and possible metastasis
image 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

image Pain, increased acidity, severe reflux, diarrhea, upper gastrointestinal tract ulcers
image Gastrinomas are often multiple (60%), malignant (60%), and metastatic (30-50%)
• Hypergastrinemia is hallmark of Zollinger-Ellison syndrome (ZES)

image Serum gastrin level of > 1,000 pg/mL is virtually diagnostic of ZES
• Prognosis

image Good with surgical resection of primary gastrinoma
image Poor if gastrinoma, liver metastases, or ulcers recur after surgery
image
(Left) Axial CECT in a 63-year-old man who presented with intractable peptic ulcer disease demonstrates hyperemia and mural thickening image of the stomach.

image
(Right) Arterial phase CECT in the same patient shows a small hypervascular gastrinoma image in the pancreatic head. It is important to distinguish this from the superior mesenteric artery image and superior mesenteric vein image.
image
(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 image, representing parathyroid adenomas or hyperplasia.

image
(Right) Abdominal CT in the same case shows marked hypervascularity and thickening of the gastric wall image. Multiple liver metastases are present image. 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

image Hypervascular pancreatic mass with multiple peptic ulcers and thickened folds
• Location

image Gastrinoma: Pancreas (75%), duodenum (15%), and liver and ovaries (10%)

– Common site: Gastrinoma triangle

image Superiorly: Cystic and common bile ducts
image Inferiorly: 2nd and 3rd parts of duodenum
image Medially: Junction of pancreatic neck and body
image Ulcers: Stomach and duodenal bulb (75%), postbulbar and jejunum (25%)

Radiographic Findings

• Barium studies: Gastric, duodenal, and proximal jejunum

image Large volume of fluid dilutes barium and compromises mucosal coating
image Markedly thickened gastric folds
image Peptic ulcers: Round or ovoid barium collections surrounded by thin or thick radiolucent rim (edematous mucosa) and radiating folds

CT Findings

• Gastrinomas

image Small or large, heterogeneous density lesion, ± cystic and necrotic areas, ± calcification
image Liver metastases are common
image Hypervascular (primary and secondary) lesions ± local or vascular invasion on arterial and portal venous phase
image Inflammatory changes in stomach, duodenum, and proximal small bowel

– Thickened gastric, duodenal, and jejunal folds
image Signs of ulcer penetration

– Wall thickening, luminal narrowing of stomach and duodenum
image Signs of ulcer perforation

– Free air in abdomen (from a duodenal or antral ulcer) or lesser sac (from a gastric ulcer)

MR Findings

• T1WI

image Hypointense pancreatic nodule on fat-saturated sequence
• T2WI

image Hyperintense on spin-echo sequence

– Both primary and metastatic tumors
• T1WI C+

image Hyperintense, hypervascular on fat-saturated delayed spin-echo sequence

Ultrasonographic Findings

• Endoscopic ultrasonography (EUS)

image Detects small gastrinomas better than CT or MR
image Usually homogeneously hypoechoic mass
• Intraoperative ultrasonography

image Detects very small tumors (75-100% sensitivity)

Angiographic Findings

• Conventional

image Hypervascular gastrinomas and metastases
image Portal venous sampling: Abnormal increase in gastrin levels after intraarterial secretin stimulation

Imaging Recommendations

• Best imaging tool

image Helical CT or MR for pancreas and possible metastasis
image EUS for additional primary sites; guides biopsy

DIFFERENTIAL DIAGNOSIS

Gastritis

• Due to Helicobacter pylori, NSAIDs, etc.
• Gastric antrum is most common site
• Double-contrast barium findings

image Thickened gastric folds
image Enlarged areae gastricae (≥ 3 mm in diameter)
• CT findings

image Circumferential antral wall thickening with submucosal edema
• Barium and CT findings may mimic ZES
• Diagnosis: Endoscopic biopsy, culture, urea breath test

Gastric Carcinoma

• Double-contrast barium findings

image Early gastric cancer

– Superficial lesion: Mucosal nodularity, ulceration, plaque-like or localized thickened gastric folds
– Indistinguishable from focal peptic ulcers of ZES
• CT findings

image Gastric carcinoma usually narrows the lumen, thickens the wall with submucosal soft tissue (not water) density
image Early gastric cancer

– Focal wall thickening with mucosal irregularity
– May simulate focal peptic ulcer disease of ZES
• Diagnosis: Endoscopic biopsy and histology

Gastric Metastases and Lymphoma

• Gastric metastases: Most common organs of origin

image Malignant melanoma, breast, lung, colon, pancreas
• Gastric lymphoma

image Stomach is most frequently involved organ in gastrointestinal (GI) tract
image Majority are non-Hodgkin lymphoma (B cell)
• Barium findings

image Malignant melanoma: Bull’s-eye or “target” lesions

– Centrally ulcerated submucosal masses
image Breast cancer metastases

– Linitis plastica or “leather bottle” appearance

image Loss of distensibility of antrum and body with thickened irregular folds
– Mucosal nodularity, ulceration, and spiculation simulate peptic ulcers of ZES
image Gastric lymphoma

– Diffusely thickened irregular folds, discrete ulcers, ulcerated submucosal masses
– Low-grade mucosa-associated lymphoid tissue (MALT) lymphoma: Confluent, variably sized nodules
• CT findings

image Markedly thickened gastric wall and mucosal folds
image Bull’s-eye, “target,” or giant cavitated lesions
image Thickened gastric folds and ulcers may simulate ZES

Extrinsic Inflammation

• Pancreatitis
• Thickened gastric wall can mimic ZES

Other Gastritides

• Crohn disease, eosinophilic gastritides
• Early gastric Crohn disease: Multiple aphthous ulcers
• Eosinophilic: Mucosal nodularity, thickened folds

PATHOLOGY

General Features

• Etiology

image Islet cell tumors are neuroendocrine tumors
image Usually due to non-β islet cell tumor (gastrinoma) of pancreas
image Gastrinomas arise from amine precursor uptake and decarboxylation (APUD) cells of islet of Langerhans
image Pathogenesis

– Gastrinoma: ↑ gastrin levels → ↑ gastric acid secretions → peptic ulcers
• Associated abnormalities

image 20-60% of cases are associated with multiple endocrine neoplasia type 1 (MEN1)

– MEN1: Tumors of pituitary, parathyroid, adrenal cortex, and pancreas
• Embryology/anatomy

image Islet cell tumor: Originates from embryonic neuroectoderm

Gross Pathologic & Surgical Features

• Tumors: Encapsulated and firm; may be cystic, necrotic, with calcifications

Microscopic Features

• Gastrinoma: Sheets of small round cells with uniform nuclei and cytoplasm
• Ulcers: Necrotic debris, zone of granulation tissue

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Pain, increased acidity, severe reflux, diarrhea, upper GI tract ulcers
• Lab data: Secretin injection test

image Paradoxical increase in serum gastrin to > 200 pg/mL above base levels in 90% of cases
• Diagnosis

image Gastrinoma and peptic ulcers on imaging
image Hypergastrinemia is hallmark of ZES

– Serum gastrin level of > 1,000 pg/mL is virtually diagnostic of ZES

Demographics

• Age

image Any age group but more common in 4th-5th decade
• Gender

image M > F
• Epidemiology

image Rare compared to tumors of exocrine pancreas
image Accounts for 0.1-1% of pancreatic tumors
image Gastrinomas are 2nd most common functioning pancreatic endocrine tumors after insulinomas
image Gastrinomas are often  multiple (60%), malignant (60%), and metastatic (30-50%)

Natural History & Prognosis

• Prognosis

image Good with surgical resection of primary gastrinoma
image Poor if gastrinoma, liver metastases, or ulcers recur after surgery
• Complications

image Gastrinoma: ↑ risk of malignancy, metastases
image Perforation of peptic ulcer

Treatment

• Medical: Protein pump inhibitors to treat gastric ulcers
• Surgical: Gastrinoma resection
• Liver metastases: Chemotherapy and hepatic artery embolization

DIAGNOSTIC CHECKLIST

Consider

• Ruling out other causes of gastric wall thickening and ulceration
• MEN1 syndrome

Image Interpretation Pearls

• Hypervascular pancreatic tumor, liver metastases with multiple ulcers and thickened folds of stomach, duodenum, and jejunum

image
(Left) Upper GI series film shows a large jejunal ulcer image resulting in a dilated duodenum due to spasm and edema. Gastric folds are thickened and the barium within the stomach is diluted and poorly adherent due to increased secretions. These are classic fluoroscopic features of ZES.
image
(Right) EUS in the same patient shows a mass image that was biopsied during the same procedure. The biopsy needle is evident image. This documented a gastrinoma, accounting for the patient’s signs and symptoms.
image
(Left) CECT in a 66-year-old man with ZES shows the gastrinoma as a small mass image with an enhancing, solid periphery and a cystic or necrotic center.

image
(Right) EUS in the same patient shows the mass (cursors) more clearly as having a cystic or necrotic center with a solid peripheral rim of tissue. EUS-guided biopsy proved this to be a benign gastrinoma.
image
(Left) Upper GI series in a 51-year-old woman with a prior gastrectomy for peptic ulcer disease, now presenting with recurrent pain, shows a marginal ulcer image at the site of the gastroenteric anastomosis. Note the radiating folds image leading to the ulcer crater.

image
(Right) Upper GI series in the same patient again shows the marginal ulcer image and the radiating folds image leading to the ulcer crater.
image
Upper GI series shows thickened gastric and duodenal folds and excess fluid in the stomach. Several duodenal ulcers are present image.

image
Upper GI series shows gastric fold thickening and excess fluid. Duodenal and jejunal strictures are present image, probably from prior ulceration.
image
Axial CECT shows thickened hypervascular gastric folds from Zollinger-Ellison syndrome.
image
Axial CECT in the arterial phase shows a small hypervascular mass image in the pancreatic head which proved to be a gastrinoma at resection.
image
Axial CECT in the portal venous phase shows a small hypodense mass image in the pancreatic head and multiple subtle liver metastases.
image
Axial CECT shows a fluid-distended intestine due to Zollinger-Ellison syndrome.
image
Upper GI series shows markedly thickened folds in the stomach, duodenum, and jejunum due to Zollinger-Ellison syndrome.
image
Axial NECT shows numerous hepatic metastases and a thick gastric wall due to Zollinger-Ellison syndrome.

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