Adrenal tumours Flashcards
Adrenal medullary tumours
Phaeochromocytoma
Neuroblastoma
Adrenal cortex tumours
Adrenocortical adenoma
Adrenocortical carcinoma
Phaeochromocytoma - definition
Derived from chromaffin cells from the adrenal medulla. Secretes cateholamines (makes too much adrenaline and noradrenaline)
Which tumour is known as the 10% tumour
Phaeohromocytoma
- 10% in children, extra-adrenal, bilateral, malignant
Phaeochromocytoma - who gets it
Runs in families
Phaeochromocytoma - clinical features
Gradual onset Triad of: hypertension, headache, sweating Postural hypotension Pallor Tachycardia Breathless Palpitations Weight loss
Phaeochromocytoma - investigations
Urinary excretion of catecholamines - cateholamine excess Urinary excretion of metabolites MRI scan - identify source of cateholamine excess MIBG scan PET scan
Phaeochromocytoma - management
alpha blocker
Beta blocker
Surgery
Chemotherapy (if malignant)
Do you give the beta blocker or alpha blocker first in management of phaeochromocytoma, and why?
Alpha blocker
- want to avoid a hypertensive crisis
Neuroblastoma - definition
Develops from immature nerve cells
Neuroblastoma - who gets it?
Children under 5
Neuroblastoma - clinical features
Abdominal pain
Mass under the skin that isn’t tender when touched
Change in bowel habits
Adrenocortical adenoma - clinical features
Incidental finding
Hormonal effects
Adrenocortical adenoma - pathology
Well circumscribed lesion
Usually small
Yellow in colour
Adrenocortical carcinoma - pathology
Large size
Heavy
Lack of clear cells
Congenital adrenal hyperplasia - definition
Primary adrenal insufficiency
Disorder of steroid synthesis
Defect in one of the enzymes necessary for cortisol synthesis
Congenital adrenal hyperplasia - which enzyme is deficient?
21-alpha hydroxylase deficiency
Congenital adrenal hyperplasia - clinical features in male
Poor weight gain
adrenal insufficiency
Congenital adrenal hyperplasia - clinical features in female
Genital ambiguity
Congenital adrenal hyperplasia - late onset clinical features
Acne
Hirsutism
Oligomenorrhoea
Precocious puberty
Congenital adrenal hyperplasia - investigations
raised testosterone levels
Congenital adrenal hyperplasia - management children
Glucocorticoid replacement
Surgical replacement
Congenital adrenal hyperplasia - management adults
Control androgen excess
