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Zollinger Ellison Syndrome

Zollinger-Ellison Syndrome

Zollinger–Ellison Syndrome is caused by a gastrin-secreting tumor and characterized by severe peptic ulceration, gastric hypersecretion and elevated serum gastrin.


Presentation

Individuals with Zollinger Ellison syndrome exhibit the following signs and symptoms:

Weight Loss
  • The presence of weight loss especially with abdominal pain, diarrhea, or heartburn is an important clue suggesting the presence of gastrinoma.[ncbi.nlm.nih.gov]
  • We present a rare case of a-12-year boy who initially presented with abdominal pain, diarrhoea, vomiting and progressive weight loss for over a two-year period before he was finally diagnosed as a case of ZES with the primary tumour in the pancreatic[ncbi.nlm.nih.gov]
  • Symptoms Symptoms of ZES include abdominal pain, diarrhea, unintended weight loss, anemia, gastrointestinal bleeding, loss of appetite, heart burn due to acid reflux, discomfort in the upper abdomen with pain and gnawing.[symptoma.com]
  • The patient presented with weight loss and abdominal distension caused by antral and duodenal stenosis due to an inveterate peptic ulcer.[ncbi.nlm.nih.gov]
  • OBJECTIVES: Zollinger-Ellison syndrome (ZES) is characterized by hypergastrinemia and gastric acid hypersecretion resulting in peptic ulcer disease, diarrhea, and weight loss.[ncbi.nlm.nih.gov]
Fatigue
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: January 30, 2019[patientslikeme.com]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019[patientslikeme.com]
  • It presents a range of unpleasant symptoms, including: Stabbing abdominal pain, due to the ulcers Fatigue and weakness Nausea, vomiting and diarrhoea Weight loss, due to a lessened ability to eat food and to the cancerous tumours, if present.[irishhealth.com]
  • The symptoms of ZES include signs of peptic ulcers: gnawing, burning pain in the abdomen; diarrhea; nausea; vomiting; fatigue; weakness; weight loss; and bleeding.[nursinglink.monster.com]
  • -anemia physical assessment findings indicative of gastric cancer -hepatomegaly -palpable epigastric mass hard, enlarged lymph nodes in left supraclavicular chain, left axilla, or umbilicus -weight loss -anemia -fatigue -pain -change in bowel habits[quizlet.com]
Pertussis
  • Furthermore, pertussis toxin pretreatment reversed the ability of somatostatin to inhibit secretin-induced increase in gastrin release and activation of adenylate cyclase.[ncbi.nlm.nih.gov]
Diarrhea
  • These results demonstrate that abdominal pain, diarrhea, and heartburn are the most common presenting symptoms in ZES and that heartburn and diarrhea are more common than previously reported.[ncbi.nlm.nih.gov]
  • […] you have severe abdominal pain that does not go away, especially if it occurs with diarrhea.[nlm.nih.gov]
  • A 41-year-old female affected by neurofibromatosis type 1 presented with a history of recurrent epigastric soreness, diarrhea, and relapsing chronic duodenal ulcer. Her serum fasting gastrin level was over 1000 pg/mL.[ncbi.nlm.nih.gov]
  • Due to this, individuals with ZES suffer from diarrhea and malabsorption. Causation of malabsorption in individuals affected by ZES actually occurs due to multiple factors.[symptoma.com]
  • A 59-year-old female presented with multifocal peptic ulcer disease and diarrhea. A fasting serum gastrin level obtained while the patient was receiving no antacid therapy was normal. A secretin stimulation test was positive.[ncbi.nlm.nih.gov]
Abdominal Pain
  • Most cases present with abdominal pain due to peptic ulceration and chronic diarrhoea not responding to general measures. The symptom complex is initially confused with other more common diseases, which lead to a delay in diagnosis.[ncbi.nlm.nih.gov]
  • These medicines also relieve abdominal pain and diarrhea. Surgery to remove a single gastrinoma may be done if the tumors have not spread to other organs. Surgery on the stomach (gastrectomy) to control acid production is rarely needed.[nlm.nih.gov]
  • A patient with intractable vomiting associated with abdominal pain and dental enamel erosion in two pregnancies had a gastrinoma. Symptoms were successfully controlled with omeprazole in a third pregnancy. All three babies were healthy.[ncbi.nlm.nih.gov]
  • The presence of weight loss especially with abdominal pain, diarrhea, or heartburn is an important clue suggesting the presence of gastrinoma.[ncbi.nlm.nih.gov]
  • We describe the case of a 53-year-old man who presented with abdominal pain, diarrhoea and hypomagnesaemia. The hypomagnesaemia proved to be due to gastrointestinal loss as urinary fractional excretion was very low, suggesting non-renal loss.[ncbi.nlm.nih.gov]
Vomiting
  • Persistent severe vomiting in pregnancy may be caused by underlying disease and should be investigated. A patient with intractable vomiting associated with abdominal pain and dental enamel erosion in two pregnancies had a gastrinoma.[ncbi.nlm.nih.gov]
  • Multivariate analysis further identified frequent vomiting and obesity as positive predictors of esophagitis, whereas Helicobacter pylori was a strong negative predictor (odds ratio, 0.16), possibly related to an elevated LESP in patients infected with[ncbi.nlm.nih.gov]
  • We present a rare case of a-12-year boy who initially presented with abdominal pain, diarrhoea, vomiting and progressive weight loss for over a two-year period before he was finally diagnosed as a case of ZES with the primary tumour in the pancreatic[ncbi.nlm.nih.gov]
  • Less than 48 hours after stopping PPIs, patient 1 developed multiple strictures of the esophagus caused by massive vomiting of gastric acid, and patient 2 presented with severe abdominal pain with intestinal microperforation from duodenal ulcers.[ncbi.nlm.nih.gov]
Nausea
  • A 28-year-old woman was admitted for further evaluation of epigastric soreness, heartburn, nausea, vomiting, diarrhea, and a significant weight loss.[ncbi.nlm.nih.gov]
  • ., anxiety, depression) Nausea is an unpleasant sensation in the stomach or the back of the throat. Nausea can be described as feeling queasy or squeamish, or as an upset stomach.[slideshare.net]
  • Common symptoms associated with gastrinoma in these patients were diarrhea (n 6), abdominal pain (n 4), and nausea/vomiting (n 4).[ncbi.nlm.nih.gov]
  • […] loss Decreased appetite Anemia When to see a doctor See your doctor if you have a persistent, burning, aching or gnawing pain in your upper abdomen, especially if you've also been experiencing nausea, vomiting and diarrhea.[fortishealthcare.com]
Epigastric Pain
  • A 60-yr-old woman presented with epigastric pain and diarrhea. Physical examination showed a large mass in the lower abdomen. Computed tomography revealed a large multilocular ovarian cyst.[ncbi.nlm.nih.gov]
  • A 51-year-old man visited the emergency department with recurrent epigastric pain.[ncbi.nlm.nih.gov]
  • pain, feelings of fullness, hiccups, tachycardia, hypotension decreased patency caused by clogged NG tube results in acute gastric dilation early manifestations of dumping syndrome *30 mins after eating -vertigo -tachycardia -syncope -sweating -pallor[quizlet.com]
  • Presentation Patients with ZES as part of the MEN1 syndrome present at an earlier age (approximately 10 years earlier) and may have relatively mild symptoms which can be overlooked. [ 5 ] Epigastric pain suggestive of peptic ulceration is common, especially[patient.info]
Flushing
  • Evaluate existence and repercussion of flushing episodes (history, clinical, heart rate and blood pressure measurement during flush). Preoperative hydration and electrolyte correction is necessary based on the laboratory investigations.[accessanesthesiology.mhmedical.com]

Workup

A complete medical history will be gathered and the signs and symptoms studied. If a family history of MEN I exist then the chances of ZES will be higher. In addition, the following diagnostic tests will be done in order to draw an appropriate conclusion:

  • Blood tests will be done to analyze the levels of serum gastrin. Elevated levels of gastrin are indicative of presence of tumors in the pancreas or duodenum. Although clinical conditions like primary hypochlorhydria and achlorhydria can elicit a false positive result in gastrin test [4]. This can also result from various other conditions, and therefore a fasting serum gastrin will yield appropriate results and is considered to be the best screening test for ZES. 
  • An endoscopy of the upper gastrointestinal tract is carried out to conduct biopsy of the tumors in the pancreas and duodenum.
  • Imaging studies such as CT scan and MRI of the abdomen would also be required. These are useful tools for localizing the tumor and evaluation of metastatic disease. These imaging modalities can survey patients presenting with ZES to diagnosed profound neoplasms in MEN 1 [5].
  • Endoscopic ultrasound is also a useful diagnostic procedure required to spot the exact location of the tumor. Individuals will be asked to fast the night before the test.
  • Surgery in the face of a negative imaging result may confirm the presence of gastrinoma tumors in almost 98% of patients with classic ZES signs, 7% of liver metastasis are incidentally found during early surgery preventing late complications and delay in treatment [6].
Gastrin Increased
  • RESULTS: A gastrin increase of 100 ng/l was found to be the most sensitive and specific criterion for a positive test.[ncbi.nlm.nih.gov]
  • Furthermore, we found that some clinical (diarrhea, duration of medical treatment), laboratory (basal acid output), and tumoral (size, extent) characteristics correlate with the serum gastrin increase post secretin and post calcium.[ncbi.nlm.nih.gov]
  • Aim(s): To investigate whether a higher dose of secretin would lead to higher serum gastrin increases, resulting in better sensitivity and specificity of the secretin stimulation test to identify Zollinger-Ellison patients.[enets.org]
  • The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of the hormone gastrin. Increased gastrin makes the stomach produce far too much acid.[mayoclinic.org]

Treatment

Removal of the tumor and suppression of excessive acid production are the basis of treatment regime. In many cases, if the tumors are successfully removed then acid suppressing medications would rarely be required. The following methods are employed to treat ZES:

  • Surgical procedures to selectively remove the gastrinoma are carried out [7]. If the tumor has spread to other parts of the body then decision to remove a single large tumor is taken. If liver is the site, then major part of the organ is removed to control the spread of the cancer.
  • Chemotherapy is employed following surgery to arrest the growth of the other tumors.
  • Medications like lanzoprazole can suppress the excessive acid in the stomach may be given to heal ulcers that have developed in the intestine and stomach [8]. However, proton pump inhibitors like lanzoprazole can cause lethal rebound acid hypersecretion state if abruptly discontinued among ZES patients [9]. For this reason, pantoprazole as an alternative may be taken as a long term maintenance with a high safety profile of up to 3 consecutive years of use [10].

Prognosis

The long-term prognosis of ZES is good. Medications to suppress the excessive acid production usually work well to relieve the symptoms. 

Complications

Timely diagnosis of the disease is necessary to prevent the onset of various secondary complications. The various complications of ZES include the following:

Etiology

Tumors usually located in the pancreas or the small intestine call for the development of ZES. These tumors are producing the hormone gastrin and are termed as gastrinomas. Gastrinomas can appear as single tumors or as a group. Of the total tumors that develop, about 60% of them are malignant in nature. As a result of this, the cancer spreads to the neighboring sites and affects the lymph nodes and the liver.

ZES can also be caused as secondary condition to individuals suffering from multiple endocrine neoplasia type 1 (MEN 1). In such individuals, in addition to pancreas, tumors develop in the endocrine system as well.

Epidemiology

It has been estimated that ZES occurs in about 0.1 to 1% individuals of the US. Due to improvised treatment profile of the disease condition, there has been a significant decrease in its morbidity and mortality rates. Due to this, as low as 5% of the individuals suffering from ZES develop complications.

Sex distribution
Age distribution

Pathophysiology

The excessive production of acid by the gastrin hormone causes the levels of acid to increase. As a result of large quantity of acid, gastrointestinal mucosal ulceration develops. Due to this, individuals with ZES suffer from diarrhea and malabsorption. Causation of malabsorption in individuals affected by ZES actually occurs due to multiple factors. These are the following:

  • Precipitation of bile salts
  • Inactivation of enzymes of the pancreas
  • Damage to the mucosa by excessive acid secretion

It has been estimated that 75% cases of ZES is sporadic in nature; while the rest 25% occur as a secondary complication to MEN I.

Prevention

So far there are no guidelines to prevent ZES. However, it is suggested that if individuals have a family history of MEN 1 then they should get tested for ZES. Genetic counseling and genetic testing of family members with history of MEN 1 is advised.

Summary

Zollinger Ellison syndrome (ZES) is characterized by increased production of the hormone gastrin. It is a disease of the gastrointestinal system, wherein presence of tumors stimulates production of excessive gastrin causing increased formation of acid in the stomach [1]. The overproduction of acid in turn leads to development of peptic ulcers. It is a rare disease condition and affects individuals in the age group of 30 to 50 years.

Patient Information

Definition

Zollinger Ellison syndrome (ZES) is characterized by excessive production of acid in the stomach due to tumors in the pancreas and duodenum. This is a rare disease condition and usually strikes individuals in age group of 30 to 50 years.

Cause

Development of a kind of tumors is the major cause of ZES. However, this disease can also present as a secondary complication to MEN 1.

Symptoms

Symptoms of ZES include abdominal pain, diarrhea, unintended weight loss, anemia, gastrointestinal bleeding, loss of appetite, heart burn due to acid reflux, discomfort in the upper abdomen with pain and gnawing.

Diagnosis

Blood tests and fasting serum gastrin tests are the primary diagnostic tools employed for detecting ZES. In addition to these, imaging studies such as CT scan and MRI are done for localizing the tumors. Endoscopic ultrasound is also necessary to spot the exact position of the tumor. Biopsy of the tumor is also carried out to study its nature.

Treatment

Treatment of ZES includes surgical removal of tumors followed by chemotherapy. Acid suppressing medications are given to suppress the excess acid production in the stomach. 

References

Article

  1. Campana D, Piscitelli L, Mazzotta E, Bonora M, Serra C, Salomone L; Corinaldesi R, Tomassetti P. Zollinger-Ellison syndrome. Diagnosis and therapy. Minerva Med. 2005; 96(3):187-206 
  2. Massaro SA, Emre SH. Metastatic gastrinoma in a pediatric patient with Zollinger-Ellison syndrome. J Pediatr Hematol Oncol. 2014; 36(1):e13-5 
  3. Ströker E, Leone L, Vandeput Y, Borbath I, Lefebvre C. Severe symptomatic hypomagnesaemia induced by the chronic use of proton pump inhibitors: a case report of a patient with Zollinger-Ellison syndrome. Acta Clin Belg. 2014; 69(1):62-5 
  4. Shah P, Singh MH, Yang YX, Metz DC. Hypochlorhydria and achlorhydria are associated with false-positive secretin stimulation testing for Zollinger-Ellison syndrome. Pancreas. 2013; 42(6):932-6 
  5. Singh MH, Fraker DL, Metz DC. Importance of surveillance for multiple endocrine neoplasia-1 and surgery in patients with sporadic Zollinger-Ellison syndrome. Clin Gastroenterol Hepatol. 2012; 10(11):1262-9 
  6. Norton JA, Fraker DL, Alexander HR, Jensen RT. Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome. Ann Surg. 2012; 256(3):509-17 
  7. Mortellaro VE, Hochwald SN, McGuigan JE, et al. Long-term results of a selective surgical approach to management of Zollinger-Ellison syndrome in patients with MEN-1. Am Surg. Aug 2009; 75(8):730-3.
  8. Wilcox CM, Seay T, Arcury JT, Mohnen J, Hirschowitz BI. Zollinger-Ellison syndrome: presentation, response to therapy, and outcome. Dig Liver Dis. 2011; 43(6):439-43 
  9. Poitras P, Gingras MH, Rehfeld JF. The Zollinger-Ellison syndrome: dangers and consequences of interrupting antisecretory treatment. Clin Gastroenterol Hepatol. 2012; 10(2):199-202
  10. Metz DC, Comer GM, Soffer E, Forsmark CE, Cryer B, Chey W, Pisegna JR. Three-year oral pantoprazole administration is effective for patients with Zollinger-Ellison syndrome and other hypersecretory conditions. Aliment Pharmacol Ther. 2006; 23(3):437-44 

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Last updated: 2019-07-11 20:24