Zollinger-Ellison syndrome overview: Difference between revisions
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{{Zollinger-Ellison syndrome}} | {{Zollinger-Ellison syndrome}} | ||
==Overview== | ==Overview== | ||
Patients with Zollinger-Ellison syndrome may experience abdominal pain and [[diarrhea]]. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment. | Patients with Zollinger-Ellison syndrome may experience [[abdominal pain]] and [[diarrhea]]. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment. | ||
Gastrinomas may occur as single tumors or as multiple, small tumors. About one-half to two-thirds of single gastrinomas are [[malignant]] tumors that most commonly spread to the [[liver]] and [[lymph node]]s near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called [[multiple endocrine neoplasia]] type I (MEN I). MEN I patients have tumors in their [[pituitary gland]] and [[parathyroid gland]]s in addition to tumors of the pancreas. | Gastrinomas may occur as single tumors or as multiple, small tumors. About one-half to two-thirds of single gastrinomas are [[malignant]] tumors that most commonly spread to the [[liver]] and [[lymph node]]s near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called [[multiple endocrine neoplasia]] type I (MEN I). MEN I patients have tumors in their [[pituitary gland]] and [[parathyroid gland]]s in addition to tumors of the pancreas. | ||
Revision as of 20:02, 2 October 2015
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]
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Overview
Patients with Zollinger-Ellison syndrome may experience abdominal pain and diarrhea. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment.
Gastrinomas may occur as single tumors or as multiple, small tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that most commonly spread to the liver and lymph nodes near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). MEN I patients have tumors in their pituitary gland and parathyroid glands in addition to tumors of the pancreas.
Historical Perspective
Zollinger-Ellison syndrome was first discovered by Zollinger RM and Ellison EH in 1955.[1]
Pathophysiology
Development of Zollinger-Ellison syndrome is the result of increased levels of gastrin due to an existing gastrinoma in the duodenum or pancreas.[1]
Causes
The cause of Zollinger-Ellison syndrome has not been identified. However, 25 to 30 percent of gastrinomas, which can cause Zollinger-Ellison syndrome, are caused by multiple endocrine neoplasia type 1 (MEN1).[2]
Differentiating Zollinger-Ellison syndrome from other Diseases
Zollinger-Ellison syndrome must be differentiated from gastric antrum syndrome[3], antral G-cell hyperplasia, peptic ulcer, gastroesophageal reflux disease (GERD), and hypergastrinemia.
Epidemiology and Demographics
The incidence of gastrinoma, which can cause Zollinger-Ellison syndrome, is approximately 0.5-2/million population/year worldwide.[4]. About 25 to 30 percent of gastrinomas are caused by multiple endocrine neoplasia type 1 (MEN1).[2]
Natural History, Complications and Prognosis
If left untreated, patients with Zollinger-Ellison syndrome may progress to develop abdominal pain, diarrhea, and heartburn[5]. Common complications of Zollinger-Ellison syndrome include upper gastrointestinal bleeding, anemia, and duodenal ulcer perforation. Prognosis is generally good, and the 5 and 10-year survival rate of patients with Zollinger-Ellison syndrome is approximately 94% and 75%, respectively.[6]
Diagnosis
History and Symptoms
Symptoms of Zollinger-Ellison syndrome include diarrhea, odynophagia, nausea, and hematemesis .
Physical Examination
Common physical examination findings of Zollinger-Ellison syndrome include epigastric tenderness[7], pallor, and jaundice.
Laboratory Findings
An elevated concentration of fasting serum gastrin level and secretin stimulation test may be helpful in the diagnosis of Zollinger-Ellison syndrome.
Abdominal CT
Abdominal CT scan may be helpful in the diagnosis of Zollinger-Ellison syndrome caused by gastrinoma. Gastrinomas are frequently multiple and often extrapancreatic (90% located in the gastrinoma triangle). Thus, they can be difficult to locate. For this reason, multiphase contrast enhanced thin slice cross-sectional imaging is ideal.[8] Findings on Abdominal CT scan suggestive of gastrinoma include clearly defined, well-enhanced mass.
Abdominal MRI
Abdominal MRI may be helpful in the diagnosis of Zollinger-Ellison syndrome caused by gastrinoma. Findings on abdominal MRI suggestive of Zollinger-Ellison syndrome include solitary lesion or multiple lesions.
Abdominal Ultrasound
Abdominal ultrasound may be helpful in the diagnosis of Zollinger-Ellison syndrome caused by gastrinoma.[9]
Other Imaging Studies
Endoscopic ultrasound and Somatostatin receptor scintigraphy (SRS) (octreotide scan) may be helpful in the diagnosis of Zollinger-Ellison syndrome caused by gastrinoma.[10]
Other Diagnostic Studies
Other diagnostic studies for Zollinger-Ellison syndrome include upper endoscopy, which demonstrates erosive esophagitis, thickened gastric folds, and antral erosions.
Treatment
Medical Therapy
Pharmacologic medical therapies for Zollinger-Ellison syndrome include proton pump inhibitors, H2-receptor antagonists, Chemotherapy, and hormonal therapy.
Surgery
The feasibility of surgery depends on the stage of gastrinoma causing Zollinger-Ellison syndrome at the time of diagnosis. However, all patients diagnosed with Zollinger-Ellison syndrome with no metastasis should be offered surgical exploration and resection.[11]
Primary Prevention
Effective measures for the primary prevention of Zollinger-Ellison syndrome include genetic testing, if family history is presents.[12]
Secondary Prevention
Secondary prevention strategies following Zollinger-Ellison syndrome include surgical resection of gastrinoma to prevent malignant trasformation and distant metastasis.[13]
References
- ↑ 1.0 1.1 Zollinger-Ellison syndrome 2015.https://en.wikipedia.org/wiki/Zollinger%E2%80%93Ellison_syndrome
- ↑ 2.0 2.1 National Institute of Diabetes and Digestive and Kidney Diseases.http://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/zollinger-ellison-syndrome/Pages/facts.aspx#causes
- ↑ SCOBIE BA, MCGILL DB, PRIESTLEY JT, ROVELSTAD RA (1964). "EXCLUDED GASTRIC ANTRUM SIMULATING THE ZOLLINGER-ELLISON SYNDROME". Gastroenterology. 47: 184–7. PMID 14201408.
- ↑ Jensen RT, Cadiot G, Brandi ML, de Herder WW, Kaltsas G, Komminoth P; et al. (2012). "ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes". Neuroendocrinology. 95 (2): 98–119. doi:10.1159/000335591. PMC 3701449. PMID 22261919.
- ↑ Roy PK, Venzon DJ, Shojamanesh H, Abou-Saif A, Peghini P, Doppman JL; et al. (2000). "Zollinger-Ellison syndrome. Clinical presentation in 261 patients". Medicine (Baltimore). 79 (6): 379–411. PMID 11144036.
- ↑ Melvin WS, Johnson JA, Sparks J, Innes JT, Ellison EC (1993). "Long-term prognosis of Zollinger-Ellison syndrome in multiple endocrine neoplasia". Surgery. 114 (6): 1183–8. PMID 7903006.
- ↑ Drake DP, Maciver AG, Atwell JD (1980). "Zollinger-Ellison syndrome in a child: medical treatment with cimetidine". Arch Dis Child. 55 (3): 226–8. PMC 1626767. PMID 7387166.
- ↑ Radiographic Features of Gastrinoma.Dr Frank Gaillard et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/gastrinoma
- ↑ Harvey, Adrian; Pasieka, Janice L.; Al-Bisher, Hassan; Dixon, Elijah (2012). "Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis". Cancers. 4 (4): 130–140. doi:10.3390/cancers4010130. ISSN 2072-6694.
- ↑ Tang, Shou-jiang; Wu, Ruonan; Bhaijee, Feriyl (2014). "Zollinger–Ellison Syndrome". Video Journal and Encyclopedia of GI Endoscopy. 1 (3–4): 666–668. doi:10.1016/j.vjgien.2013.06.005. ISSN 2212-0971.
- ↑ Norton JA, Fraker DL, Alexander HR, Venzon DJ, Doppman JL, Serrano J; et al. (1999). "Surgery to cure the Zollinger-Ellison syndrome". N Engl J Med. 341 (9): 635–44. doi:10.1056/NEJM199908263410902. PMID 10460814.
- ↑ Harvey A, Pasieka JL, Al-Bisher H, Dixon E (2012). "Primary hepatic gastrinoma causing zollinger-ellison syndrome: a rare and challenging diagnosis". Cancers (Basel). 4 (1): 130–40. doi:10.3390/cancers4010130. PMC 3722648. PMID 24213231.
- ↑ Epelboym I, Mazeh H (2014). "Zollinger-Ellison syndrome: classical considerations and current controversies". Oncologist. 19 (1): 44–50. doi:10.1634/theoncologist.2013-0369. PMC 3903066. PMID 24319020.