Phaeochromocytoma Flashcards
what are the symptoms of Phaeochromocytoma?
Hypertension
(persistent in 70%)
Paroxysmal attacks
headache
sweating
palpitations
tremor
pallor
anxiety/fear
what is the 10% tumour?
The ‘10% tumour’
10% extra-adrenal
10% malignant
10% multiple
10% hyperglycaemia
what genes are associated with Phaeochromocytoma?
30% inherited origin
Genes
RET, VHL,NF 1, SDH(A,B,C,D), SDHAF2 or SDH5 gene, TMEM127 gene
Familial syndromes
MEN-2, VHL, NF1, PGL
what conditions are associated with MEN II?
Phaechromocytoma
Medullary Thyroid Cancer
Parathyroid adenoma
what conditions are associated with Von Hippel-Landau syndrome?
Phaechromocytoma
Cerebral and retinal angiomatosis
Pancreatic and renal cysts
Epidydymal cystadenomas
what conditions are associated with MEN III?
Phaechromocytoma
Medullary Thyroid Cancer
Mucocutaneous Syndromes
Mutiple Neuromas ( Von Recklinghausen’s disease)
Marfanoid habitus
Hypertrophied corneal nerves
to prepare a patient with Phaeochromocytoma for surgery what must be done?
what is the management plan for a clinically inapparent adrenal mass?
Why does a deficit in 21-hydroxylase cause adrenal hyperplasia???
Lack of 21-hydroxylase inhibits synthesis of cortisol.
This removes the negative feedback on ACTH and CRH release.
Increased ACTH secretion is responsible for enlargement of adrenal glands.
Negative feedback of ACTH on CRH synthesis remains.
what is the prevalence of Congenital adrenal hyperplasia?
> 90% cases due to 21-hydroxylase deficiency
what happens in severe cases of congenital hyperplasia?
neonatal salt-losing crisis
ambiguous genitalia (girls)
what incomplete defects can arise from congenital adrenal hyperplasia?
pseudo-precocious puberty (boys)
hirsutism (women)
how does each adrenal disorder affect each layer of the adrenal cortex?
