Treatment

Medical Care

• Hypercalcemia: For patients with type 1 multiple endocrine neoplasia (MEN) who have hypercalcemia, surgery is the treatment of choice, including removal of 3.5 parathyroid glands.
• Gastrinoma: The current treatment consists of PPIs to reduce acid hypersecretion.
• Insulinoma: Surgery is the therapy of choice. Unresectable tumors are treated with diazoxide.
• Glucagonoma: Glucagonomas are surgically removed.
• VIPoma: Octreotide controls symptoms (diarrhea) in 80% of patients; however, surgical tumor removal should be attempted.
• Prolactinoma: Prolactinomas are treated with dopamine agonists, such as bromocriptine or cabergoline.
• GH-producing pituitary tumor: These tumors are treated by transsphenoidal surgery; in rare instances, medical therapy with a GH receptor antagonist is recommended.

Surgical Care

• Type 1 multiple endocrine neoplasia with hyperparathyroidism: In patients with type 1 multiple endocrine neoplasia (MEN) who have hyperparathyroidism, surgery is the treatment of choice if any of the following conditions are present:
  • Serum albumin–adjusted serum calcium level is more than 1 mg/dL of the upper limit of the reference range
  • Kidney stones
  • PTH-induced bone disease with a T score of -2.5
  • 24-hour urinary calcium excretion of more than 400 mg
• Type 1 multiple endocrine neoplasia with ZES
  • In patients with type 1 MEN with ZES, parathyroid surgery is indicated even in mild forms of hypercalcemia because serum calcium levels in the reference range are often associated with lower serum gastrin levels and consecutively lower gastric acid secretion (high calcium stimulates gastrin in those patients).
  • Removal of 3.5-4 parathyroid glands controls hypercalcemia. If 4 glands are removed, immediate autograft of parathyroid tissue into the musculature of the nondominant arm is indicated.
  • Some authors recommend taking careful operative notes and marking the residual parathyroid tissue with clips because reoperation in patients with type 1 MEN is likely.
• Gastrinoma
  • The role of surgery in ZES and type 1 MEN remains controversial because cure is only occasionally achieved. Most tumors are multicentric, raising the possibility of recurrence. Surgery may be indicated in patients with positive findings on imaging studies and no distant metastases.
  • Gastrinomas are found in the duodenal wall, in the pancreas, or in lymph nodes.

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