screening for MEN-II and von Hippel-Lindau syndrome
Last reviewed 01/2018
Every patient with a phaeochromocytoma should be screened for both MEN-II and von Hippel-Lindau syndrome (VHS).
Screening is important because:
- up to a quarter of patients will have either von Hippel Lindau or MEN type 2
- it allows identification of life-threatening problems:
- VHS - haemangioma of the brain and renal cancer
- MEN-II - medullary carcinoma of the thyroid
- of associated morbidity e.g. retinal angiomas can cause loss of vision in VHS
- it allows subsequent screening of first-degree relatives
Screening should entail:
- pentagastrium test - measure of calcitonin
- measurement of serum parathyroid hormone
- ophthalmoscopy - retinal angiomas
- MRI of the brain - haemangioblastomas
- CT of the kidneys and pancreas - retinal cysts and pancreatic cysts
- ultrasonography of the testes - epididymal cystadenoma