Pediatric Multiple Endocrine Neoplasia Medication

Updated: Oct 11, 2017
  • Author: Alicia Diaz-Thomas, MD, MPH; Chief Editor: Robert P Hoffman, MD  more...
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Medication

Medication Summary

Pharmacologic therapy for multiple endocrine neoplasia (MEN) is directed toward specific endocrine syndromes associated with the disorder. Patients with type 1 MEN and ZES need lifelong acid inhibition with PPIs.

As previously mentioned, somatostatin therapy is indicated in patients with acromegaly in whom surgery did not achieve complete tumor removal. One third of patients should experience tumor shrinkage, and up to two thirds of patients should achieve normalization of IGF-1 levels.

Although surgery is the therapy of choice for insulinomas, unresectable tumors are treated with the hyperglycemic agent diazoxide. There are some recent studies investigating the use of long acing somatostatin ligands in the treatment of unresectable or refractory insulinoma.  

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Somatostatin analogs

Class Summary

Octreotide acetate acts similarly to the natural hormone somatostatin by suppressing peptide secretion from gastroenteropancreatic tumors.

Octreotide acetate (Sandostatin)

Octreotide acetate acts primarily on somatostatin receptor subtypes II and V. It inhibits GH secretion and has other endocrine and nonendocrine effects, including inhibition of glucagon, VIP, and gastrointestinal (GI) peptides. Octreotide acetate controls diarrhea in 80% of patients. Progressive increases in dosage may be necessary.

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Somatostatin Analogs

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Proton Pump Inhibitors

Class Summary

Gastric acid secretion with PPIs is mandatory to prevent complications of gastric acid hypersecretion. PPIs are safe and cause no adverse effects, even after long-term use. The goal of therapy with these agents is to reduce the basal acid output to less than 10mEq/h 1 hour before the next dose in patients without previous acid-reducing gastric surgery, and to less than 5mEq/h in patients with previous acid-reducing gastric surgery.

Omeprazole (Prilosec)

Omeprazole is a substituted benzimidazole that suppresses acid secretion by specifically inhibiting the H+/K+ adenosine triphosphatase (ATPase) at the secretory surface of the parietal cell.

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Hypoglycemia Antidotes

Class Summary

These agents inhibit insulin release from the tumor.

Diazoxide (Proglycem)

Diazoxide binds to the sulfonylurea receptor (SUR1) of the pancreatic beta cell, inhibiting insulin secretion. The oral form opens K adenosine triphosphate (ATP) channels and inhibits insulin secretion. Diazoxide increases a patient's blood glucose level within 1 hour by inhibiting insulin release from the patient's insulinoma. Unlike rapid IV administration of diazoxide, oral administration of the drug is not antihypertensive.

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Antineoplastics, Tyrosine Kinase Inhibitor

Cabozantinib (Cabometyx, Cometriq)

Can be used in refractory medullary thyroid cancer 

Vandetanib (Caprelsa)

Can be used in refractory medullary thyroid cancer

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