Phaeochromocytoma Flashcards
What is phaeochromocytoma?
tumour arising from catecholamine-producing chromaffin cells of the adrenal medulla
What are phaeochromocytoma associated with?
✶ MEN 2a/b
✶ VHL
✶ NF type 1
What are the different types of phaeochromocytoma?
✶ RARE
✶ 10% extra-adrenal
✶ 10% malignant
✶ 10% bilateral
What are features of phaeochromocytoma?
- Hypertension
- Palpitations
- Sweating
- Anxiety
What investigations are done for paheochromocytoma?
- BP
2. Urinary
What would BP show in phaeochromocytoma?
Dangerously high (systolic >200) and in a young person
What would urinary show in phaeochromocytoma?
24-hour urinary metanephrines
No longer use catecholamines
What is the management of phaeochromocytoma?
- Alpha blockade
- Beta blockade
- Surgical excision
What is involved in alpha blockade?
phenoxybenzamide to reduce BP
What does a beta blockade do?
prevent reflex tachycardia
What are complications of phaeochromocytoma?
- Hypertensive crisis
- Cardiac arrhythmia
- Death
What 3 primary tumours are associated with MEN 2?
- medullary thyroid cancer
- parathyroid tumors
- phaeochromocytoma
What is VHL?
Von Hippel-Lindau syndrome (VHL) is a hereditary condition associated with tumors arising in multiple organs
What is neurofibromatosis 1 caused by?
gene mutation on chromosome 17 which encodes neurofibromin and affects around 1 in 4,000
What is neurofibromatosis 1 caused by?
- Café au lait spots
- axillary freckles
- peripheral neurofibromas
- phaeos
