zollinger ellison syndrome

zollinger ellison syndrome

It is also known as GASTRINOMA

it is a disease in which tumor cause the stomach to produce too much acid.

1. Tumor of  G cells
2. most common site of G cells is stomach but gastrinoma occur most commonly in DUODENUM > PANCREAS > STOMACH
3. also can present in a rare site as ovary , testis , heart

Tumor lead to gastrin ++++ which leads to ulcers

characterized by hypergastrinemia and hyperchlorhydria (recurrent peptic ulcer)

• 25% cases have association with MEN 1 (multiple endocrine neoplasia type 1) can be pituitary adenoma, parathyroid adenoma , pancreatic adenoma
• most common MEN 1 overall pancreatic adenoma is zollinger ellison syndrome

clinical features:

usual age of presentation 30 - 50 years (female > males)

1. abdominal pain (epigastric pain) - hunger pain present
2. diarrhea(due to excess acid production inactivate pancreatic enzymes)
3. GERD (cause reflux esophagitis, retrosternal pain)
4. past medical history kidney stone

NOTE:

• very important to confirm diagnosis
• patient on PPI(proton pump inhibitor) can has increase gastrin level in blood
• so alone increase gastrin level can't make the diagnosis ZES

WORK UP:

ENDOSCOPE

• upper GI  Endoscopy: Multiple DU/Giant DU
• Breath urea test : negative - rules out H.pylori
• Urease test : negative - rules out H.pylori

- ca2+/PTH/PRL/Pancreatic polypeptide levels tested to see association with MEN 1 : chr  11q13

= MEN 1 Involved -pituitary adenoma

                                    -parathyroid adenoma

                                  -pancreatic adenoma

IOC - SECRETIN STUDY

• check for fasting gastrin level > 1000 pg/ml(increase 10times)
• Blood acid output > 15meq/hr
• BAO/MAO (Basal area output/maximal acid output) > 0.6

TUMOR LOCALIZATION

Tumor can be present in hypothetical gastrinoma triangle

1. confluence/junction of common hepatic duct and common bile duct = A
2. Head and body of pancreas dissected = C
3. between D2 and D3 (D = DUODENUM) = B

TREATMENT:

1. DOC - PPI (will not control diarrhea component)
2. somatostatin analog - Lanreotide
3. surgical resection : if tumor >1.5 - 2 cms(CT/MRI)
4. increase calcium lead to acid increase from stomach 

parathyroid adenoma - TC - 99/sestamibi scan - surgical resection

1.   metastasis liver : Hepatic resection