This document discusses Zollinger-Ellison syndrome (ZES), a condition caused by tumors that secrete high levels of the hormone gastrin and lead to stomach acid hypersecretion. It describes the signs, symptoms and various diagnostic tests used to detect and localize gastrinomas, including fasting serum gastrin tests, gastric acid secretion tests, somatostatin receptor scintigraphy, CT, MRI, EUS, and others. Treatment options discussed include proton pump inhibitors to control acid levels, surgical removal of localized tumors, and other approaches for advanced cases such as chemotherapy or targeted therapies.

7. Stomach

8. Small intestine

9. Pancreas

10. MEDICALTERMINOLOGY

12. PATHOPHYSIOLOGY

14. multiple endocrine
neoplasia type 1 (MEN1).

15. SIGNS AND SYMPTOMS

16. CLINICAL MANIFESTATIONS

17. LABORATORYPROCEDURES

18. Fasting Serum Gastrin

19. Gastric acid secretory tests
• The patient receives an injection of the hormone gastrin or
insulin. A tube is put through the nose or throat into the
stomach and samples are taken from the stomach and
sent to a laboratory for testing.Also called stomach acid
stimulation test and stomach acid test.
• Gastric pH less than 2.0 in the presence of a large gastric
volume (>140 mL over 1 h in patients without prior gastric
acid–reducing surgery) is highly suggestive of ZES.

21. Serum Chromogranin A (Serum CGA)

22. RADIOLOGICPROCEDURE

24. SOMATOSTATIN RECEPTOR SCINTIGRAPHY
(SRS)
• A type of radionuclide scan used to find carcinoid and other
types of tumors. Radioactive octreotide, a drug similar to
somatostatin, is injected into a vein and travels through the
bloodstream.
• Imaging method of choice because of its high sensitivity in
detecting primary or metastatic lesions in ZES

25. • Able to differentiate between gastrinomas and coexisting
carcinoid tumors of the stomach in patients with MEN-1-
associated ZES
• Disadvantage: Does not give reliable data about tumor size
and exact location (eg duodenum vs pancreatic head)

27. Fig. 3 a, b. SRS 24 h after i.v. injection of 111In-pentatreotide in a patient with ZES
and MEN 1.aAnterior view of the abdomen.The scintiscan confirmed a tumour of
the head of the pancreas (open arrow) and three lymph nodes (arrow-heads) in the
duodenopancreatic area, as previously shown by CT. Also, tracer uptake was
heterogeneous in the left lobe of the liver (curved arrow), whereas conventional
methods, including MRI, showed a normal liver, b. Anterior view of the abdomen
obtained 1 year later in the same patient.The abnormalities previously found in the
duodenopancreatic area are unchanged. However, hot spots are now clearly
distinguishable in the liver (arrows).

28. COMPUTED TOMOGRAPHY (CT)
• Helical CT is highly sensitive for the detection of primary
pancreatic tumors and hepatic metastases
• May provide additional information about tumor size and
location

29. Figure 1. Computed axial tomography scan from a patient
with gastrinoma metastatic to the liver.The location of the
metastatic gastrinoma is indicated by the arrows.

30. Figure 2. Computed axial tomography scan demonstrating the
appearance of a gastrinoma in the pancreatic head.The location
of the gastrinoma is labeled "tumor" and indicated by the arrow.

31. Figure 3. CT scan of the abdomen with
contrast enhancement, arterial-phase
image. Note the brightly enhancing mass
in the wall of the duodenum medially
(arrow).Courtesy of J. Kevin Smith, MD,
PhD.

32. Magnetic Resonance Imaging (MRI)
• Has a high sensitivity for detecting hepatic
metastases
• Disadvantage: Less sensitive for detection of
primary pancreatic tumors

33. Endoscopic Ultrasound (EUS)

34. Figure 3 :
EUS showing hypertrophic gastric rugae (a) and neuroendocrine
tumor in the pancreatic neck (b), respectively, as indicated by
arrows.

35. Angiography
• The role of angiography and selective arterial stimulation in
the localization of gastrinomas is less clear. The sensitivity of
angiography ranges from 40%–57%.
• The utility of angiography rests on the highly vascular nature
of gastrinomas.
• . A venous catheter is inserted into the right or middle hepatic
vein. After injection of a standard dose of secretin, gastrin
levels are measured at regular intervals.[

37. UPPER-GI SERIES

39. Image from an upper gastrointestinal
series.A 5-cm ulcer crater in the lesser
curve of the stomach is depicted face.
The filling defects in the ulcer crater are
caused by a blood clot from recent
bleeding.

40. MANAGEMENTANDMEDICATIONS

41. Surgical Management:
• In cases where the gastrinoma is localised to the pancreas
or small intestine without any evidence of spread to other
organs, surgical removal of the tumour can cure the
disease.
• While initially, if tumour is too big the surgical therapy
proposed was total gastrectomy with the aim of removing
the high levels of gastrin from the target organ.

42. •Management of symptoms:

43. PROTON PUMP INHIBITORS (PPIS)
• Most effective drugs for controlling gastric acid hypersecretion in
ZES patients
These drugs, which include :
• dexlansoprazole (Dexilant ),
• esomeprazole (Nexium), lansoprazole (Prevacid),
• omeprazole (Prilosec, Zegerid),
• pantoprazole (Protonix),
• rabeprazole (Aciphex),

44. •Management of tumour progression:

45. In some cases where the tumour spread is aggressive
alternative treatments are may be used and these
include:
•chemotherapy
•tyrosine kinase inhibitors or mTor inhibitors
•peptide targeted radionuclide therapy (PRRT)
•trans-arterial embolisation/chemoembolization.

46. REFERENCES

47. • https://www.yourhormones.info/endocrine-conditions/gastrinoma/
• https://www.hopkinsmedicine.org/health/conditions-and-diseases/zollingerellison-
syndrome#:~:text=ZES%20is%20caused%20by%20tumors,of%20your%20stomach%20and%20in
testines
• http://nlp.case.edu/public/data/TargetedToxicity_JCOFullText/SVM_text_classifier_training/trainin
g/negative/0_5.html
• Berna, Marc J.; Hoffmann, K. Martin; Serrano, Jose; Gibril, Fathia; Jensen, RobertT. (2006). Serum
Gastrin in Zollinger-Ellison Syndrome. Medicine, 85(6), 295–
330. doi:10.1097/01.md.0000236956.74128.76
• https://emedicine.medscape.com/article/183555-workup#c6