Zollinger-Ellison syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Aravind Reddy Kothagadi M.B.B.S[2] Mohamad Alkateb, MBBCh [3]

Overview

Pharmacologic medical therapies for Zollinger-Ellison syndrome include proton pump inhibitor (PPI), H2-receptor antagonists, chemotherapy, and hormonal therapy.

Medical Therapy

  • Pharmacotherapy of Zollinger-Ellison syndrome (ZES) includes aspects of management such as medical control of acid hypersecretion, diagnosis, localization and treatment directed at the gastrinoma.
  • Widespread use of PPI for multiple GI complaints is making the diagnosis of ZES more difficult and is delaying the diagnosis.
  • Certain aspects of ZES require modifications of standard anti-secretory treatment and are discussed (pregnancy, parenteral therapy, complicated disease)
  • Patients with advanced disease require treatments directed against the gastrinoma, a number of which are recently shown effective or promising including new chemotherapy regimens, molecular targeted therapies, biotherapies, and peptide-radioreceptor therapy.
  • As the widespread use of pharmacotherapy has become more prevalent, the management of ZES has transformed from a surgical therapy to medical therapy which has been observed to play a major role. [1]
  • Medical management is to treat symptoms and prevent complications from peptic ulcer disease. The preferred medical therapy is the use of high doses of proton pump inhibitors. PPI’s are preferred over H2 receptor blockers due to their higher potency and longer duration of action. [2]

Pharmacotherapy for Zollinger-Ellison syndrome may include the following: [1] [3] [4]

Hormonal Therapy

  • In patients who are not suitable for surgery or in patients with widespread metastasis, conservative management with PPIs is also recommended.[2]
  • In patients with metastatic disease a limited efficacy has been observed with current treatment modalities. Chemotherapy may be advised for patients with widespread metastasis. The first-line treatment suggested is combined therapy with streptozotocin and 5-fluorouracil or doxorubicin. However, these treatments have been shown to result in limited responses and considerable toxicity.[2]

References

  1. 1.0 1.1 Ito T, Igarashi H, Uehara H, Jensen RT (2013). "Pharmacotherapy of Zollinger-Ellison syndrome". Expert Opin Pharmacother. 14 (3): 307–21. doi:10.1517/14656566.2013.767332. PMC 3580316. PMID 23363383.
  2. 2.0 2.1 2.2 Cingam S, Karanchi H. PMID 28722872. Missing or empty |title= (help)
  3. Metz DC, Comer GM, Soffer E, Forsmark CE, Cryer B, Chey W; et al. (2006). "Three-year oral pantoprazole administration is effective for patients with Zollinger-Ellison syndrome and other hypersecretory conditions". Aliment Pharmacol Ther. 23 (3): 437–44. doi:10.1111/j.1365-2036.2006.02762.x. PMID 16423003.
  4. Hirschowitz BI, Simmons J, Mohnen J (2005). "Clinical outcome using lansoprazole in acid hypersecretors with and without Zollinger-Ellison syndrome: a 13-year prospective study". Clin Gastroenterol Hepatol. 3 (1): 39–48. PMID 15645403.
  5. Saijo F, Naito H, Funayama Y, Fukushima K, Shibata C, Hashimoto A; et al. (2003). "Octreotide in control of multiple liver metastases from gastrinoma". J Gastroenterol. 38 (9): 905–8. doi:10.1007/s00535-002-1170-8. PMID 14564638.
  6. Burgess JR, Greenaway TM, Parameswaran V, Shepherd JJ (1999). "Octreotide improves biochemical, radiologic, and symptomatic indices of gastroenteropancreatic neoplasia in patients with multiple endocrine neoplasia type 1 (MEN-1). Implications for an integrated model of MEN-1 tumorigenesis". Cancer. 86 (10): 2154–9. PMID 10570446.
  7. Shojamanesh H, Gibril F, Louie A, Ojeaburu JV, Bashir S, Abou-Saif A; et al. (2002). "Prospective study of the antitumor efficacy of long-term octreotide treatment in patients with progressive metastatic gastrinoma". Cancer. 94 (2): 331–43. doi:10.1002/cncr.10195. PMID 11900219.

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