Multiple Endocrine Neoplasia, Type 2 Follow-up

  • Author: Melanie L Richards, MD, MPHE; Chief Editor: George T Griffing, MD   more...
 
Updated: Feb 19, 2010
 

Further Inpatient Care

  • Admit patients for testing and surgical intervention.
  • Patients should be monitored on a life-long basis for evidence of recurrent disease. After an initial follow-up visit, patients may be evaluated at 6 months, then yearly if they are asymptomatic. During these evaluations, patients should undergo physical examination, 24-hour urine catecholamine, metanephrine and vanillylmandelic acid, CEA level, calcitonin, and serum calcium testing.
    • If recurrent hypercalcemia is suggested, consider patients for repeat cervical exploration.
    • If pheochromocytoma is suggested, evaluate patients for surgical resection. This tumor is likely in the remaining contralateral adrenal, although workup should include a CT scan and MIBG scan to evaluate for recurrence in the resected area or an extra-adrenal site. Recurrences in the resected area are more common if a subtotal adrenalectomy had been performed initially. The management of calcitonin/CEA elevations has been controversial. Resect any palpable cervical disease.
    • Some practitioners have advocated routine cervical ultrasonography with exploration for any evidence of recurrence. Many patients remain asymptomatic with elevated calcitonin levels for 20 years or longer.
    • The 5- and 10-year survival rates in patients with medullary thyroid carcinoma and MEN 2A are approximately 90% and 75%, respectively.
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Further Outpatient Care

  • Monitor patients for recurrence of medullary thyroid carcinoma with calcitonin, CEA, and +/- provocative calcitonin testing.
  • Perform annual screening for hyperparathyroidism with serum calcium and PTH levels in MEN 2A patients.
  • Obtain urinary catecholamine levels on an annual basis to assess for pheochromocytoma.
  • Carefully monitor medication dosage and adverse effects.
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Inpatient & Outpatient Medications

  • Hormone replacement following total thyroidectomy and bilateral adrenalectomy is necessary. Patients who develop postoperative hypoparathyroidism need supplemental calcium and/or vitamin D.
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Transfer

  • Transfer patients for surgical intervention if necessary.
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Deterrence/Prevention

  • Prophylactic thyroidectomy prevents medullary thyroid carcinoma.
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Complications

  • Hypercalcemia
  • Chronic constipation
  • Hypertensive episode
  • Recurrence of medullary thyroid carcinoma
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Prognosis

  • Early treatment of medullary thyroid carcinoma can prevent death.
  • Careful monitoring for pheochromocytomas can decrease the chance of hypertensive episodes.
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Patient Education

  • Adhering to a surveillance program lessens disease complications.
  • Order genetic counseling to discuss gene testing and reproductive options.
  • For excellent patient education resources, visit eMedicine's Endocrine System Center. Also, see eMedicine's patient education article Thyroid Problems.
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Contributor Information and Disclosures
Author

Melanie L Richards, MD, MPHE  Associate Professor, Department of Surgery, Mayo Clinic

Melanie L Richards, MD, MPHE is a member of the following medical societies: American Association of Endocrine Surgeons, American College of Surgeons, International Association of Endocrine Surgeons, Southwestern Surgical Congress, and Western Surgical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Suzanne M Carter, MS  Senior Genetic Counselor, Associate, Department of Obstetrics and Gynecology, Division of Reproductive Genetics, Montefiore Medical Center, Albert Einstein College of Medicine

Suzanne M Carter, MS is a member of the following medical societies: American Bar Association

Disclosure: Nothing to disclose.

Susan J Gross, MD, FRCS(C), FACOG, FACMG  Codirector, Division of Reproduction Genetics, Associate Professor, Department of Obstetrics and Gynecology, Albert Einstein College of Medicine

Susan J Gross, MD, FRCS(C), FACOG, FACMG is a member of the following medical societies: American College of Medical Genetics, American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, American Medical Association, American Society of Human Genetics, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Ghassem Pourmotabbed, MD†  Former Associate Professor, Department of Internal Medicine, Division of Endocrinology and Metabolism, University of Tennessee School of Medicine and Health Science Center

Ghassem Pourmotabbed, MD† is a member of the following medical societies: American Diabetes Association, American Federation for Medical Research, and Endocrine Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Romesh Khardori, MD, PhD  Professor and Director, Division of Endocrinology, Metabolism, and Molecular Medicine, Southern Illinois University School of Medicine

Romesh Khardori, MD, PhD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society of Andrology, Endocrine Society, and Illinois State Medical Society

Disclosure: Nothing to disclose.

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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