Adrenal - Tumours Flashcards
Adrenocortical tumours may occur in children and young adults in association with which genetic condition?
Li-Fraumeni syndrome
What is the only definite criteria for malignancy in adrenocortical tumours?
Metastases
What is the commonest solid organ malignancy in those aged < 5 years?
Neuroblastoma
What is the median age of onset of a neuroblastoma?
20 months
How does a neuroblastoma most commonly present?
Abdominal swelling
How should neuroblastomas be treated?
Surgical excision and chemotherapy
Where are phaeochromocytomas most likely to be located?
The adrenal medulla
What do phaeochromocytomas produce?
Catecholamines
Extra-adrenal phaeochromocytomas are most likely to be found where?
At the aortic bifurcation
Describe the 10% rule associated with phaechromocytoma?
10% malignant, 10% extra-adrenal, 10% bilateral, 10% familial
What would make you suspicious that a phaeochromocytoma was related to a genetic condition?
If it occurred in a young individual and/or was bilateral
10% of phaeochromocytomas are related to a hereditary syndrome. What may these be?
MEN2A, MEN2B, Von-Hippel-Lindau syndrome, neurofibromatosis
What is the classic triad of phaeochromocytoma?
Episodic headache, sweating and tachycardia
Describe the hypertension which occurs with a phaeochromocytoma?
Often labile (comes on with stress, exercise, palpation of the tumour…) and may be postural
What happens to the WCC in those with a phaeochromocytoma?
Raised
