MEN

Question 1

Multiple endocrine neoplasia (MEN) is inherited as an autosomal ____ disorder

Answer 1

Dominant

MEN are DOMINANT

Question 2

MEN type I associated cancers?

Answer 2

3 P’s

Parathyroid (95%)
Pituitary (60%)
Pancreas (50%) e.g. insulinoma, gastrinoma (>recurrent peptic ulceration)

Also: adrenal and thyroid

Question 3

most common presentation of MEN type I?

Answer 3

hypercalcaemia (hyperparathyroidism)

Question 4

gene associated with MEN I?

Answer 4

MEN1 gene

Question 5

MEN type IIa associated cancers?

Answer 5

Medullary thyroid cancer (70%)

2 P’s:
Parathyroid
Phaeochromocytoma

Question 6

gene associated with MEN IIa?

Answer 6

RET oncogene

Question 7

MEN type IIb associated cancers?

Answer 7

Medullary thyroid cancer

1 P:
Phaeochromocytoma
Marfinoid body habitus
Neuromas

Question 8

gene associated with MEN IIb?

Answer 8

RET oncogene

Question 9

what is Phaeochromocytoma

Answer 9

rare catecholamine secreting tumour

Question 10

10% rule of Phaeochromocytoma?

Answer 10

10% malignant
10% B/L
10% extraadrenal (most commonly organ of Zuckerkandl)

Question 11

features of Phaeochromocytoma?

Answer 11

episodic:
hypertension (90%)
headaches
palpitation
sweating
anxiety

Question 12

test for Phaeochromocytoma?

Answer 12

24hr urinary collection of metanephrines (97% sensitivity)

NB: has replaced 24h urinary collection of catecholamines

Question 13

management of Phaeochromocytoma?

Answer 13

Surgery is the definitive management
must be stabilised first
- alpha-blocker (e.g. phenoxybenzamine) before a
- beta-blocker (e.g. propranolol)

(A before B)

Question 14

All MEN II tend to have what cancer as a presenting feature

Answer 14

medullary thyroid cancer