pheochromocytoma Flashcards
define pheochromocytoma?
catecholamine - producing tumours that usually arise from chromaffin cells of the adrenal medulla
NOT ADENOMA- adenomas are cushing’s syndrome, conns, testosterone producing = tumours arising from cortex
outline the different types of tumours for pheochromocytoma?
extra- adrenal in about 10%-> extra-adrenal phaemochromocytomas are referred to as paragangliomas
10% bilateral
10% are malignant
outline the risk factors for pheochromocytoma?
Multiple endocrine neoplasia (MEN) syndrome type 2A and B
Von Hippel-Lindau (VHL) disease
neurofibromatosis type 1 (NF1)
succinate dehydrogenase (SDH) subunit B, C, and D gene mutations
outline the epidemiology of pheochromocytoma?
- RARE
* <0.2% of cases of hypertensive patients
what are the signs of pheochromocytoma?
• Hypertension • Postural hypotension • Pallor • Tachycardia • Fever Weight loss
what are the symptoms of pheochromocytoma?
- PAROXYSMAL episodes – comes in attacking manner
- Headache (due to malignant hypertension)
- Sweating
• Cardiorespiratory Symptoms ○ Palpitations ○ Chest pain ○ Dyspnoea • GI Symptoms ○ Epigastric pain ○ Nausea ○ Constipation • Neuropsychiatric Symptoms ○ Weakness ○ Tremor - Anxiety
what are the signs of pheochromocytoma?
hypertension postural hypotension pallor tachycardia fever weight loss
outline the investigations for pheochromocytoma?
- 24 hr urine collection - check for catecholamine levels (and check for fractionated metanephrine/ vandillylmandelic acid levels)
○ NOTE: metanephrines are metabolites of adrenaline - Plasma free metanephrines and catecholamines
○ Most labs won’t be able to perform this
○ But acc more sensitive than urine - Genetic testing – done if under 50 mostly
• Screen for associated conditions
• Serum calcium and potassium + CBC
• Tumour localisation (MRI or CT adrenal protocol not just CT abdoandpelvis)
• Gallium-68 PET/CT scan
I-MIBG scintigraphy (another way of visualising the tumour) - but really very rarely done nowadays
Outline the treatment for pheochromocytoma?
- Functional tests come back
- alpha blockade
- send for imaging
- beta-blocker (if the pt develops a reflex tacchycardia so not all patients will require a betablockade)
- Monitor the BP and slowly increase the alpha-blockade (few weeks)
- finally surgical excision of adrenal gland
○ Retroperitoneal keyhole surgery
▪ Issue with other abdo viscera if done through the front
▪ Less pain for pt and shorter length of stay
○ Anterior and lateral approach keyhole – put pt on the side so all the other viscera fall away
▪ Some patients may not be able to lie on the front
▪ Most easy to convert into open so would do this if it looks very big and malignant
○ Open surgery through front or back (with back remove 12th rib)
- Follow up
○ Surgery went
○ Annual follow up (serum or urine)
Genetic testing if under 50
describe the different methods of surgical intervention for pheochromocytoma ?
Retroperitoneal keyhole surgery
- Issue with other abdo viscera if done through the front
- Less pain for pt and shorter length of stay
Anterior and lateral approach keyhole – put pt on the side so all the other viscera fall away
- Some patients may not be able to lie on the front
- Most easy to convert into open so would do this if it looks very big and malignant
Open surgery through front or back (with back remove 12th rib)