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Sipple's syndrome

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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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.


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