Multiple Endocrine Neoplasia Type 2 Overview
Learn About Multiple Endocrine Neoplasia Type 2
View Main Condition: Neural Crest Tumor
Multiple endocrine neoplasia, type II (MEN II) is a disorder passed down through families in which one or more of the endocrine glands are overactive or form a tumor. Endocrine glands most commonly involved include:
- Adrenal gland (about half the time)
- Parathyroid gland (20% of the time)
- Thyroid gland (almost all the time)
Multiple endocrine neoplasia, type I (MEN I) is a related condition.
Sipple syndrome; MEN II; Pheochromocytoma - MEN II; Thyroid cancer - pheochromocytoma; Parathyroid cancer - pheochromocytoma
The cause of MEN II is a defect in a gene called RET. This defect causes many tumors to appear in the same person, but not necessarily at the same time.
Involvement of the adrenal gland is most often with a tumor called a pheochromocytoma.
Involvement of the thyroid gland is most often with a tumor called medullary carcinoma of the thyroid.
Tumors in the thyroid, adrenal, or parathyroid glands may occur years apart.
The disorder may occur at any age, and affects men and women equally. The main risk factor is a family history of MEN II.
There are two subtypes of MEN II. They are MEN IIa and IIb. MEN IIb is less common.
The symptoms may vary. However, they are similar to those of:
- Medullary carcinoma of the thyroid
- Pheochromocytoma
- Parathyroid adenoma
- Parathyroid hyperplasia
Surgery is needed to remove a pheochromocytoma, which can be life threatening due to the hormones it makes.
For medullary carcinoma of the thyroid, the thyroid gland and surrounding lymph nodes must be totally removed. Thyroid hormone replacement therapy is given after surgery.
If a child is known to carry the RET gene mutation, surgery to remove the thyroid before it becomes cancerous is considered. This should be discussed with a physician who is very familiar with this condition. It would be done at an early age (before age 5) in people with known MEN IIa, and before age 6 months in people with MEN IIb.
Osu Surgery, LLC
John Phay is a Surgical Oncologist and a General Surgeon in Columbus, Ohio. Dr. Phay and is rated as an Advanced provider by MediFind in the treatment of Multiple Endocrine Neoplasia Type 2. His top areas of expertise are Adrenocortical Carcinoma, Thyroid Cancer, Adrenal Cancer, Parathyroidectomy, and Thyroidectomy. Dr. Phay is currently accepting new patients.
Richard Kloos is an Endocrinologist in Columbus, Ohio. Dr. Kloos and is rated as an Advanced provider by MediFind in the treatment of Multiple Endocrine Neoplasia Type 2. His top areas of expertise are Thyroid Nodule, Thyroid Cancer, Medullary Thyroid Carcinoma, Follicular Thyroid Cancer, and Thyroidectomy.
Christopher Breuer is a Pediatric Surgeon and a General Surgeon in Columbus, Ohio. Dr. Breuer and is rated as an Experienced provider by MediFind in the treatment of Multiple Endocrine Neoplasia Type 2. His top areas of expertise are Chediak-Higashi Syndrome, Multiple Endocrine Neoplasia Type 2, Pulmonary Atresia, Kidney Transplant, and Thyroidectomy. Dr. Breuer is currently accepting new patients.
Pheochromocytoma is most often not cancerous (benign). Medullary carcinoma of the thyroid is a very aggressive and potentially fatal cancer, but early diagnosis and surgery can often lead to a cure. Surgery does not cure the underlying MEN II.
The spread of cancerous cells is a possible complication.
Contact your provider if you notice symptoms of MEN II or if someone in your family receives such a diagnosis.
Screening close relatives of people with MEN II may lead to early detection of the syndrome and related cancers. This may allow for steps to prevent complications.
Background: - Medullary thyroid cancer (MTC) is a rare cancer of the thyroid gland. In children and adults, it is often part of a condition called Multiple Endocrine Neoplasia 2 (MEN2). MEN2 is usually caused by a genetic mutation, and it can cause a number of problems in addition to MTC. These problems include adrenal gland tumors, hormone changes, and problems with the bones and other organs. Not much is kn...
Background: Bariatric surgery is gaining in popularity. While it's health benefits are undisputed, the older malabsorptive bariatric procedures (Roux-in-Y gastric bypass - RYGB and biliopancreatic diversion - BPD) are associated with an increased risk of fractures and falls as early as 3-5 years after surgery. Sleeve gastrectomy - SG is now the most performed bariatric procedure. Although SG does not cause ma...
Published Date: March 31, 2024
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Gild ML, Tsang V, Clifton-Bligh RJ, Robinson BG. Multiple endocrine neoplasia types 2 and 3, and medullary thyroid carcinoma. In: Robertson RP, ed. DeGroot's Endocrinology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 134.
National Comprehensive Cancer Network website. Clinical practice guidelines in oncology (NCCN guidelines): neuroendocrine and adrenal tumors. Version 1.2023. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Updated August 2, 2023. Accessed May 7, 2024.
Newey PJ, Thakker RV. Multiple endocrine neoplasia. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 42.
Nieman LK, Spiegel AM. Polyglandular disorders. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 212.