Sipple's syndrome
Last reviewed 01/2018
This is the most common variant of MEN-II and is characterised by:
- multiple phaeochromocytomas in the adrenal medulla
- medullary carcinoma of the thyroid
- less commonly, parathyroid hyperplasia or adenoma
Medullary thyroid tumours are present in over 90% of cases. They are usually an early sign in MEN-IIA and commonly, are very slow growing. Mortality and morbidity may be reduced significantly by screening for serum calcitonin in combination with pentagastrin stimulation.
Phaeochromocytomas are present in about 50% of cases and are a major cause of death. They should be excluded or treated in any patient with MEN-IIA.