Adrenal malignancies Flashcards
Neuroendocrine tumours - Name 2
in the context of endocrine:
Pheochromocytoma
Carcinoid tumour
What is pheochromocytoma and what is the associated gene
Adrenal medulla malignancy of chromaffin cells - causing excess of adrenaline and noradrenaline (catecholamines)
MEN 2 gene
How may pheochromocytoma present
SNS Overload:
Flushing
Episodic headache
Anxiety
Sweating
Pallor
Tachycardic
Palpitations
HTN
Pheochromcytoma DX
Urine/Plasma Metanephrines (catecholamines breakdown product)
If +
Adrenal CT - Abdo CT/MRI
How do you treat pheochromocytoma?
Targeting adrenergic receptors:
Alpha receptor blocker - phenoxybenzamine
Beta receptors blocker - Atenolol
Give pre operation - Adrenalectomy
What is carcinoid tumour?
Poorly malignant tumour of enterochromaffin cells (GI, ileum/appendinx) - secreting excess serotonin (5HT)
How would carcinoid tumour present?
Only neoplastic cells - ASX
once mets to Liver - carcinoid syndrome:
Diagnostic Sx:
diarrhoea
flushing
palpitations
abdominal cramps
telangiectasia
signs of right heart failure
Murmur - tricuspid incompetence
SOB
Carcinoid tumour Dx
Urine 5HIAA (serotonin METABOLITE)
GS - Chromogranin A marker (+ octreotide scan )
Liver US - Mets
Abdo CT/MRI
What is octreotide scan
An octreotide scan is a type of SPECT scintigraphy used to find carcinoid, pancreatic neuroendocrine tumors, and to localize sarcoidosis
Octreotide is a somatostatin analogue.
This is is radiolabelled
The radioactive octreotide attaches to tumour cells that have receptors for somatostatin . A gamma camera detects the radioactive octreotide, and makes pictures showing where the tumor cells are in the body, typically by a SPECT technique.
carcinoid tumour TX
Surgical excise tumour
+ Octreotide
What is a complication of carcinoid tumour
carcinoid crisis - present acutly and medical emergency
carcinoid crisis, which involves severe flushing, breathlessness and a changes in your blood pressure.
TX-doubel octreotide
