What is Multiple Endocrine Neoplasia II (MEN II) (Sipple’s syndrome)

Multiple Endocrine Neoplasia II (MEN II) or Sipple’s syndrome occurs when patients develop a type of thyroid cancer accompanied by recurring cancer of the adrenal glands.

One type of this disease (MEN IIa) is also associated with overgrowth (hyperplasia) of the parathyroid gland.

Statistics on Multiple Endocrine Neoplasia II (MEN II) (Sipple’s syndrome)

The cause of MEN II is genetic – a mutation in a gene called RET. Multiple tumors may appear in the same person, but not necessarily at the same time. The disorder may occur at any age, and affects men and women equally. The main risk factor is a family history of MEN II.

Risk Factors for Multiple Endocrine Neoplasia II (MEN II) (Sipple’s syndrome)

The cause of MEN II is genetic – a mutation in a gene called RET. Multiple tumors may appear in the same person, but not necessarily at the same time. The adrenal tumor is a pheochromocytoma and the thyroid tumor is a medullary carcinoma of the thyroid.

The disorder may occur at any age, and afflicts men and women on an equal basis. The main risk factor is considered to be a family history of MEN II.

How is Multiple Endocrine Neoplasia II (MEN II) (Sipple’s syndrome) Diagnosed?

Symptoms include; severe headache, palpitations, rapid heart rate, sweating, chest pain, abdominal pain, nervousness, irritability, weight loss, diarrhoea, cough, cough with blood, fatigue, back pain, increased urine output, increased thirst, appetite loss, nausea, muscular weakness, depression, personality changes

Note: The symptoms may vary, but are consistent with those of pheochromocytoma, medullary carcinoma of the thyroid, and sometimes hyperparathyroidism.

Prognosis of Multiple Endocrine Neoplasia II (MEN II) (Sipple’s syndrome)

Pheochromocytoma is usually benign (not cancer), but the accompanying medullary carcinoma of the thyroid that characterizes this condition is a very aggressive and potentially fatal cancer. Nonetheless, early diagnosis and surgery can often lead to cure.

Mean survival is six to seven decades.

How is Multiple Endocrine Neoplasia II (MEN II) (Sipple’s syndrome) Treated?

Surgery is needed to remove both the medullary carcinoma of the thyroid and the pheochromocytoma. Medullary carcinoma of the thyroid must be treated with total removal of the thyroid gland and removal of surrounding lymph nodes. Hormone replacement therapy is given after surgery.

Multiple Endocrine Neoplasia II (MEN II) (Sipple’s syndrome) References

[1] Ballinger, A. Patchett, S., Saunders’ Pocket Essentials of Clinical Medicine 2nd ed. W.B. Saunders, 2000
[2] Longmore M, Wilkinson I, Torok E. OXFORD HANDBOOK OF CLINICAL MEDICINE. Oxford Universtiy Press. 2001
[3] Robbins, S.L., Cotran, R.S., Kumar, V. Robbins Pathologic Basis of Disease 5th ed. W.B. Saunders 1995.