eMedicine Specialties > Pediatrics: General Medicine > Endocrinology

Multiple Endocrine Neoplasia: Follow-up

Author: Robert J Ferry Jr, MD, Chief, Division of Pediatric Endocrinology and Diabetes, Le Bonheur Children's Medical Center, University of Tennessee Health Science Center at Memphis and St Jude Children's Research Hospital; Lieutenant Colonel (Medical Corps), 162nd Area Support Medical Company, Army National Guard
Coauthor(s): Klaus Radebold, MD, PhD, Research Associate, Department of Surgery, Yale University School of Medicine; Christian A Koch, MD, PhD, FACP, FACE, Professor and Director, Division of Endocrinology, University of Mississippi Medical Center; George P Chrousos, MD, FAAP, MACP, MACE, Professor and Chair, Department of Pediatrics, Athens University Medical School
Contributor Information and Disclosures

Updated: Jun 12, 2008

Follow-up

Further Inpatient Care

  • After parathyroid surgery, monitor serum ionized calcium and magnesium levels. Transient hypoparathyroidism frequently develops and requires calcium and vitamin D supplementation. In rare cases, hungry-bone syndrome may ensue, with rapid declines in serum calcium and magnesium levels.
  • Somatostatin therapy is indicated particularly in patients with acromegaly in whom surgery did not achieve complete tumor removal. Tumor shrinkage can be expected in one third of patients, normalization of IGF-1 levels can be expected in as many as two thirds.
  • Pregnancy may ensue with treatment that includes cabergoline or bromocriptine in patients with prolactinoma.

Further Outpatient Care

  • In MTC, measure serum calcitonin levels every 6-12 months after surgery. Elevated serum levels in the neck region indicate recurrence that possibly requires further surgery. However, micrometastases often cannot be visualized by using current imaging techniques.
  • The resection of one endocrine tumor does not exclude a second tumor, even after an interval of several years. Patients need lifelong surveillance, as do their offspring.
  • Pheochromocytomas are monitored with blood pressure and plasma free metanephrines as well as 24-hour urinary catecholamines, including fractionated metanephrine measurements, to exclude recurrence.

Inpatient & Outpatient Medications

  • Patients with type 1 multiple endocrine neoplasia (MEN) and ZES need lifelong acid inhibition with PPIs.
  • Chemotherapy with streptozocin and dacarbazine may reduce the size of nonoperable neuroendocrine tumors.

Deterrence/Prevention

  • Because early therapy substantially improves the patient's prognosis, screening is of paramount importance in all forms of multiple endocrine neoplasia (MEN).
  • Risk factors include known MEN, a positive family history of MEN, ZES, ganglioneuromas, cutaneous neuromas, a marfanoid somatic phenotype, parathyroid hyperplasia, multicentric medullary carcinoma of the thyroid, and multicentric or bilateral pheochromocytomas.
  • Screening tests include the following measurements based on the type of MEN:
    • Type 1 multiple endocrine neoplasia - Serum calcium and intact PTH, prolactin, plasma chromogranin A, fasting gastrin, fasting glucose and insulin, and free IGF-I determinations
    • Type 2A multiple endocrine neoplasia -RET germline mutation testing and determinations of plasma calcitonin, plasma free metanephrines and 24-hour urinary catecholamines (including fractionated metanephrines), and serum calcium levels
    • Type 2B multiple endocrine neoplasia -RET germline mutation testing and determinations of serum calcitonin, plasma free metanephrines, and 24-hour urinary catecholamines including fractionated metanephrines
  • If test results are in the reference range on 3 occasions and if the patient is older than 35 years, he or she can be declared a noncarrier. Offspring of noncarriers do not require testing. Mutation analysis of the MENIN gene enables the identification of people carrying a germline mutation.

Prognosis

  • Type 1 multiple endocrine neoplasia
    • The prognosis is generally good in the presence of discrete parathyroid and pancreatic islet disease or pituitary adenoma.
    • Pancreatic islet cell carcinoma and carcinoids are slowly progressive.
    • Patients with gastrinoma in type 1 MEN may have a prognosis better than that of patients with the sporadic form of the disease.
  • Type 2A multiple endocrine neoplasia: The prognosis depends on the stage of medullary thyroid cancer and is generally good after prophylactic thyroidectomy.
  • Type 2B multiple endocrine neoplasia
    • The prognosis for patients with type 2B MEN is worse than for patients with type 2A MEN because tumors, such as MTCs, are relatively aggressive, resulting in a 10-year survival rate of 50%.
    • Therefore, individuals with an RET germline mutation in exon 16 should undergo early prophylactic thyroidectomy and screening for pheochromocytomas.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • In patients with type 1 multiple endocrine neoplasia (MEN) with ZES who have hypercalcemia, perform parathyroidectomy before abdominal tumoral resection. Lowering serum calcium levels to the reference range may also normalize elevated gastric acid secretion.
  • Pheochromocytomas in patients with type 2 MEN may be bilateral. Explore both adrenal glands during surgery.
  • Patients with a pheochromocytoma and MTC should receive appropriate preparation with alpha-blocker therapy. Then, the pheochromocytoma should be removed first to avoid life-threatening complications (eg, hypertensive crisis, arrhythmias) caused by the adrenal tumor. Laparoscopic adrenalectomy has now become a routine procedure.
 


More on Multiple Endocrine Neoplasia

Overview: Multiple Endocrine Neoplasia
Differential Diagnoses & Workup: Multiple Endocrine Neoplasia
Treatment & Medication: Multiple Endocrine Neoplasia
Follow-up: Multiple Endocrine Neoplasia
References
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Further Reading

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Keywords

multiple endocrine neoplasia, MEN syndrome, MEN 1, MEN 2A, MEN 2B, Wermer syndrome, Wermer's syndrome, Sipple syndrome, Sipple's syndrome, multiple endocrine adenopathy, MEA, pluriglandular syndrome, Carney complex, vasoactive intestinal peptide tumor, VIPoma, pancreatic polypeptide–producing tumor, PPoma, medullary thyroid carcinoma, MTC, gastrinoma, insulinoma, glucagonoma, prolactinoma, somatotropinoma, corticotropinoma, nonfunctioning tumors, lipomas, angiofibromas, pheochromocytoma, hyperparathyroidism, parathyroid gland hyperplasia, thyroid cancer, mucosal neuroma, medullated corneal nerve fibers, marfanoid habitus, peptic ulcer disease, coronary heart disease, hypercalcemia, stroke, heart failure, Zollinger-Ellison syndrome, ZES, Hirschsprung disease

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Contributor Information and Disclosures

Author

Robert J Ferry Jr, MD, Chief, Division of Pediatric Endocrinology and Diabetes, Le Bonheur Children's Medical Center, University of Tennessee Health Science Center at Memphis and St Jude Children's Research Hospital; Lieutenant Colonel (Medical Corps), 162nd Area Support Medical Company, Army National Guard
Robert J Ferry Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, American Medical Association, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, Society for Pediatric Research, and Texas Pediatric Society
Disclosure: Nutropin Speakers Bureau Honoraria Speaking and teaching

Coauthor(s)

Klaus Radebold, MD, PhD, Research Associate, Department of Surgery, Yale University School of Medicine
Klaus Radebold, MD, PhD is a member of the following medical societies: American Gastroenterological Association and New York Academy of Sciences
Disclosure: Nothing to disclose.

Christian A Koch, MD, PhD, FACP, FACE, Professor and Director, Division of Endocrinology, University of Mississippi Medical Center
Christian A Koch, MD, PhD, FACP, FACE is a member of the following medical societies: American Academy of Neurology, American Association of Clinical Endocrinologists, American College of Clinical Endocrinologists, American College of Physicians-American Society of Internal Medicine, American Society for Clinical Pharmacology and Therapeutics, American Society for Dermatologic Surgery, Endocrine Society, and German Diabetes Society
Disclosure: Nothing to disclose.

George P Chrousos, MD, FAAP, MACP, MACE, Professor and Chair, Department of Pediatrics, Athens University Medical School
George P Chrousos, MD, FAAP, MACP, MACE is a member of the following medical societies: American Academy of Pediatrics, American College of Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Arlan L Rosenbloom, MD, Adjunct Distinguished Service Professor Emeritus of Pediatrics, University of Florida; Fellow of the American Academy of Pediatrics; Fellow of the American College of Epidemiology
Arlan L Rosenbloom, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Epidemiology, American Pediatric Society, Endocrine Society, Florida Pediatric Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Lynne Lipton Levitsky, MD, Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor, Department of Pediatrics, Harvard University Medical School
Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD, Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and Arkansas Children's Hospital
Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research
Disclosure: Genentech, Inc. Honoraria Speaking and teaching; Pfiser, Inc. Honoraria Consulting

 
 
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