Medication Summary
Many hormonal abnormalities may be expressed in patients with multiple endocrine neoplasia type 1 (MEN1). Medications that may be used are outlined below.
Somatostatin analogues
Class Summary
These drugs suppress peptide secretion of gastroenteropancreatic tumors or growth hormone – producing tumors. They have also been reported to relieve pain from spinal metastasis.
Octreotide (Sandostatin)
Octreotide acts primarily on somatostatin receptor subtypes II and V. It inhibits growth hormone secretion and has a multitude of other endocrine and nonendocrine effects, including inhibition of glucagon, vasoactive intestinal peptide, and GI peptides.
Proton pump inhibitors
Class Summary
Proton pump inhibitors inhibit gastric acid secretion by inhibition of the H+/K+ -ATP-ase enzyme system in the gastric parietal cells. Esomeprazole (Nexium), omeprazole (Prilosec), pantoprazole (Protonix), rabeprazole (AcipHex), and lansoprazole (Prevacid) are available.
Omeprazole (Prilosec)
Proton pump inhibitors effectively block the H+, K+-ATPase of the parietal cell at the secretory surface and inhibit acid secretion, which is required in MEN1-associated gastrinomas. The goal is to reduce the basal acid output to less than 10 mEq/h 1 hour prior to the next dose in patients without previous acid-reducing gastric surgery and to less than 5 mEq/h in patients with previous acid-reducing gastric surgery.
Esomeprazole (Nexium)
Esomeprazole is an S-isomer of omeprazole. It inhibits gastric acid secretion by inhibiting H+/K+-ATPase enzyme system at the secretory surface of gastric parietal cells.
Pantoprazole (Protonix)
Pantoprazole suppresses gastric acid secretion by specifically inhibiting the H+/K+-ATPase enzyme system at the secretory surface of gastric parietal cells. Use of the intravenous preparation has only been studied for short-term use (ie, 7-10 d).
Rabeprazole sodium (AcipHex)
Rabeprazole sodium suppresses gastric acid secretion by specifically inhibiting the H+/K+-ATPase enzyme system at the secretory surface of gastric parietal cells.
Lansoprazole (Prevacid)
Rabeprazole sodium suppresses gastric acid secretion by specifically inhibiting the H+/K+-ATPase enzyme system at the secretory surface of gastric parietal cells.
Dopamine agonists
Class Summary
Dopamine agonists are the treatment of choice in prolactinomas. In growth hormone producing pituitary tumors, they are usually added to somatostatin analogues if complete remission has not been achieved. They have modest effects if used as a single agent for acromegaly. Bromocriptine (Parlodel) and cabergoline (Dostinex) are available.
Bromocriptine (Parlodel)
Bromocriptine is a dopamine agonist that reduces pituitary production of prolactin and may shrink prolactinomas. It may be an alternative for treatment of acromegaly, but adverse effects at high dose may limit applicability.
Cabergoline (Dostinex)
Cabergoline is a long-acting dopamine receptor agonist with high affinity for D2 receptors and low affinity for D1 receptors. It inhibits prolactin secretion. Prolactin secretion by the anterior pituitary predominates under hypothalamic inhibitory control exerted through dopamine.
Antihypoglycemic agents
Class Summary
These agents increase blood glucose by inhibiting pancreatic insulin release and possibly through an extrapancreatic effect.
Diazoxide (Hyperstat, Proglycem)
The inhibitory effect of diazoxide in insulinoma may be effective in 90% of patients. The remaining 10% may not respond or tolerate the drug. Treat adverse effects with hydrochlorothiazide. Hyperglycemic effects start within 1 hour and usually last a maximum of 8 hours with normal renal function. In patients not responsive to diazoxide, somatostatin may be indicated.
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Multiple endocrine neoplasia type 1 (MEN1). Sagittal (left image) and coronal (right image), T1-weighted magnetic resonance images of the brain in a patient with MEN1 show a pituitary macroadenoma (arrows).
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Multiple endocrine neoplasia type 1 (MEN1). Indium-111 (111In) octreotide scan in a patient with MEN1 demonstrates abnormal activity in the pituitary macroadenoma (curved arrow), parathyroid adenoma (straight arrow), and gastrinoma metastases throughout the abdomen (arrowheads).
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Multiple endocrine neoplasia type 1 (MEN1). Technetium-99m sestamibi scan (99mTc MIBI) in a patient with MEN1 demonstrates persistent abnormal activity of the inferior right parathyroid gland that is consistent with an adenoma.
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Multiple endocrine neoplasia type 1 (MEN1). Computed tomography (CT) scan of the pancreas in a patient with MEN1 and a gastrinoma shows a pancreatic head mass (large, white arrow), as well as a low-attenuating lesion in the liver (small, black arrowhead) that indicates metastases. Note the calcifications of the right renal medullary pyramids (medullary nephrocalcinosis; black arrows) in this nonenhanced CT scan.
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Multiple endocrine neoplasia type 1 (MEN1). Endoscopic ultrasonogram in a patient with an insulinoma. The hypoechoic neoplasm (arrows) is seen in the body of the pancreas anterior to the splenic vein (SV). (From: Rosch T, Lightdale CJ, Botet JF, et al. Localization of pancreatic endocrine tumors by endoscopic ultrasonography. N Engl J Med. Jun 25 1992;326(26):1721-6.)
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Multiple endocrine neoplasia type 1 (MEN1). Computed tomography (CT) scan image with oral and intravenous contrast in a patient with biochemical evidence of insulinoma. The 3-cm contrast-enhancing neoplasm (arrow) is seen in the tail of the pancreas (P) posterior to the stomach (S) (From: Yeo CJ. Islet cell tumors of the pancreas. In: Niederhuber JE, ed. Current Therapy in Oncology. St. Louis, Mo: Mosby-Year Book; 1993: 272.)
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Multiple endocrine neoplasia type 1 (MEN1). Anteroposterior radiographic view of the right hand in a patient with MEN1 and primary hyperparathyroidism shows subperiosteal bone resorption along the radial aspects of the middle phalanges (arrows).
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Multiple endocrine neoplasia type 1 (MEN1). Bilateral, anteroposterior radiographic views of the hands in a patient with MEN1 and primary hyperparathyroidism show subperiosteal bone resorption along the radial aspects of the middle phalanges.
Tables
Tumor |
Age at which to begin screening (yr) |
Blood tests (annual) |
Imaging studies |
Insulinoma |
5 |
Fasting glucose, insulin |
– |
Anterior pituitary |
5 |
Prolactin, IGF-I |
MRI (every 3 yr) |
Parathyroid |
8 |
Calcium, PTH |
– |
Other pancreatic neuroendocrine tumors |
< 10 |
Chromogranin-A; pancreatic polypeptide, glucagon, VIP |
MRI, CT, or EUS (annually) |
Adrenal |
< 10 |
Only in patients with symptoms or signs of a functioning tumor and/or tumor >1 cm identified on an imaging study |
MRI or CT (annually with pancreatic imaging) |
Thymic and bronchial carcinoid |
15 |
None |
CT or MRI (every 1-2 yr) |
Gastrinoma |
20 |
Gastrin (± gastric pH) |
– |
CT = computed tomography; EUS = endoscopic ultrasound; IGF-1 = Insulin-like growth factor 1; ( MRI = magnetic resonance imaging; PTH = parathyroid hormone; VIP = vasoactive intestinal peptide Adapted from Thakker RV, et al. Clinical Practice Guidelines for Multiple Endocrine Neoplasia Type 1 (MEN1). J Clin Endocrinol Metab. 2012 Sep. 97(9):2990-3011. [1] |
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