eMedicine Specialties > Endocrinology > Multiple Endocrine Disease and Miscellaneous Endocrine Disease

Wermer Syndrome (MEN Type 1): Follow-up

Author: Irina Lendel, MD, Clinical Instructor in Endocrinology, Division of Endocrinology, Diabetes, and Metabolism, Milton S Hershey Medical Center
Coauthor(s): James M Hammond, MD, Distinguished Professor of Medicine, Penn State University College of Medicine, Milton S Hershey Medical Center; Klaus Radebold, MD, PhD, Research Associate, Department of Surgery, Yale University School of Medicine
Contributor Information and Disclosures

Updated: May 14, 2009

Follow-up

Further Inpatient Care

  • Monitor calcium levels after parathyroid surgery. Hypoparathyroidism may develop in 10-25% of patients. Recurrent hyperparathyroidism is also possible.
  • Patients with acromegaly and residual tumor after surgery may respond favorably to somatostatin, which causes tumor shrinkage in approximately one third of cases.

Further Outpatient Care

  • Hyperparathyroidism in patients with MEN 1 recurs in approximately 50% of patients within 10 years. Possible factors include the following:
    • Parathyroid cell growth can be an ongoing process.
    • A serum factor (eg, fibroblast growth factor) may stimulate endothelial cell growth in the parathyroid glands.
    • Hyperplasia results from expansion of multiple cell clones and not from a single cell type as in parathyroid adenomas.
  • In patients with moderately elevated calcium levels, surgery may be postponed while the patient is assessed periodically, including serum creatinine, urine calcium, and bone density measurements.
  • Carcinoid tumors may be associated with MEN 1. Perform careful endoscopic monitoring of fundic argyrophil cells or appropriate imaging at yearly intervals for known primary lesions.
  • Hepatic artery embolization can be used to manage pancreatic tumors that have metastasized to the liver.

Inpatient & Outpatient Medications

  • In patients with MEN 1 and gastrinoma, acid inhibition with proton pump inhibitors must be continued for life.
  • Chemotherapy with streptozocin and dacarbazine may reduce tumor size in unresectable disease.

Transfer

  • Because MEN 1 is a rare neuroendocrine disease, diagnosis and treatment should be performed in centers specializing in endocrinology.

Prognosis

  • The prognosis in patients with MEN 1 and gastrinoma is considered better than in those with ZES alone.
  • Whether surgery with tumor resection improves survival remains controversial. In one French study, complete resection of the primary tumor did not reduce the incidence of subsequent liver metastases.
 


More on Wermer Syndrome (MEN Type 1)

Overview: Wermer Syndrome (MEN Type 1)
Differential Diagnoses & Workup: Wermer Syndrome (MEN Type 1)
Treatment & Medication: Wermer Syndrome (MEN Type 1)
Follow-up: Wermer Syndrome (MEN Type 1)
Multimedia: Wermer Syndrome (MEN Type 1)
References
Further Reading
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References

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Further Reading

Related eMedicine topics:
Gastrinoma
Glucagonoma
Hyperparathyroidism [Emergency Medicine]
Hyperparathyroidism [Endocrinology]
Hyperparathyroidism [Otolaryngology and Facial Plastic Surgery]
Hyperparathyroidism [Pediatrics: General Medicine]
Hyperparathyroidism, Primary
Insulinoma
Multiple Endocrine Neoplasia
Multiple Endocrine Neoplasia Type 1
Multiple Endocrine Neoplasia, Type 2
Neoplasms of the Endocrine Pancreas
Pancreas, Islet Cell Tumors
Prolactinoma
Zollinger-Ellison Syndrome [Gastroenterology]
Zollinger-Ellison Syndrome [Pediatrics: General Medicine]
Zollinger-Ellison Syndrome [Radiology]

Clinical guidelines:
Intraoperative parathyroid hormone. Laboratory medicine practice guidelines: evidence-based practice for point-of-care testing. National Academy of Clinical Biochemistry - Professional Association.  2006.  15 pages.  NGC:005645

Procedure guideline for parathyroid scintigraphy. Society of Nuclear Medicine, Inc - Medical Specialty Society.  1999 Feb (revised 2004 Jun).  6 pages.  NGC:004256

Clinical studies:
Studies of Inherited Diseases of Metabolism

Treatment of Zollinger-Ellison Syndrome With Prevacid

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Keywords

multiple endocrine neoplasia type 1, MEN 1, parathyroid, hyperparathyroidism, pituitary tumor, MEN 1 syndrome, MEN 2 syndrome, MEN 2a, MEN 2b, prolactinoma, insulinoma, pituitary tumors, pancreatic tumor, pancreatic tumors, endocrine disorder, endocrine disease, endocrine diseases, glucagonoma, islet cell tumor, pluriglandular syndrome, multiple endocrine adenopathy, MEA, Wermer's syndrome, parathyroid tumor, pancreatic islet cell tumor, pituitary hyperplasia, pituitary tumor formation, pancreas tumor, pancreatic polypeptide tumors, PPomas, gastrinomas, VIPoma, vasoactive intestinal polypeptide tumor, Zollinger-Ellison syndrome, ZES, Cushing syndrome, Cushing’s syndrome, Sipple syndrome

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Contributor Information and Disclosures

Author

Irina Lendel, MD, Clinical Instructor in Endocrinology, Division of Endocrinology, Diabetes, and Metabolism, Milton S Hershey Medical Center
Disclosure: Nothing to disclose.

Coauthor(s)

James M Hammond, MD, Distinguished Professor of Medicine, Penn State University College of Medicine, Milton S Hershey Medical Center
James M Hammond, MD is a member of the following medical societies: Alpha Omega Alpha, American Diabetes Association, American Federation for Clinical Research, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Phi Beta Kappa, and Society for the Study of Reproduction
Disclosure: Nothing to disclose.

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.

Medical Editor

Frederick H Ziel, MD, Associate Professor of Medicine, David Geffen School of Medicine at UCLA; Physician-In-Charge, Endocrinology/Diabetes Center, Director of Medical Education, Kaiser Permanente Woodland Hills; Chair of Endocrinology, Co-Chair of Diabetes Complete Care Program, Southern California Permanente Medical Group
Frederick H Ziel, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society for Bone and Mineral Research, California Medical Association, Endocrine Society, and International Society for Clinical Densitometry
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS, Professor of Medicine (Endocrinology, Adj), Johns Hopkins School of Medicine; Affiliate Research Professor, Bioinformatics and Computational Biology Program, School of Computational Sciences, George Mason University; Principal, C/A Informatics, LLC
Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Nutrition, American College of Physician Executives, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, American Society for Bone and Mineral Research, American Society of Law, Medicine & Ethics, Endocrine Society, and International Society for Clinical Densitometry
Disclosure: Nothing to disclose.

CME Editor

Mark Cooper, MBBS, PhD, FRACP, Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University
Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD, Professor of Medicine, St Louis University School of Medicine
George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

 
 
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