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Flashcards in Adrenal - Tumours Deck (20)
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1

Adrenocortical tumours may occur in children and young adults in association with which genetic condition?

Li-Fraumeni syndrome

2

What is the only definite criteria for malignancy in adrenocortical tumours?

Metastases

3

What is the commonest solid organ malignancy in those aged < 5 years?

Neuroblastoma

4

What is the median age of onset of a neuroblastoma?

20 months

5

How does a neuroblastoma most commonly present?

Abdominal swelling

6

How should neuroblastomas be treated?

Surgical excision and chemotherapy

7

Where are phaeochromocytomas most likely to be located?

The adrenal medulla

8

What do phaeochromocytomas produce?

Catecholamines

9

Extra-adrenal phaeochromocytomas are most likely to be found where?

At the aortic bifurcation

10

Describe the 10% rule associated with phaechromocytoma?

10% malignant, 10% extra-adrenal, 10% bilateral, 10% familial

11

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

12

10% of phaeochromocytomas are related to a hereditary syndrome. What may these be?

MEN2A, MEN2B, Von-Hippel-Lindau syndrome, neurofibromatosis

13

What is the classic triad of phaeochromocytoma?

Episodic headache, sweating and tachycardia

14

Describe the hypertension which occurs with a phaeochromocytoma?

Often labile (comes on with stress, exercise, palpation of the tumour...) and may be postural

15

What happens to the WCC in those with a phaeochromocytoma?

Raised

16

What is the diagnostic investigation of choice for a phaeochromocytoma?

Plasma and 3x free urinary metanephrines

17

What investigations can be used to identify the location of a phaeochromocytoma?

Abdominal CT/MRI or MIBG chromaffin-seeking isotope scan

18

How is a phaeochromocytoma treated definitively?

Surgical excision

19

What treatment must be given before surgery for a phaeochromocytoma?

Alpha blocker (e.g. phenoxybenzamine) followed by beta blocker (e.g. propranolol)

20

Why must alpha blockers be given before beta blockers in the treatment of a phaeochromocytoma?

Beta blockers alone can worsen hypertension