Multiple Endocrine Neoplasia Flashcards
1
Q
How is MEN inherited?
A
Autosomal dominant
2
Q
What are the 3 types of MEN?
A
- Type 1 - 3 P’s
- Type IIa - 2P’s
- Type IIb - 1P
3
Q
How does MEN type 1 present (Think 3 Ps)
A
- Parathyroid tumours (95%) –> hypercalcaemia is the most common presentation.
- Pituitary tumours (70%) –> e.g. prolactinomas.
-
Pancreatic tumours (50%)
- Insulinomas –> Low blood sugar.
- Gastrinomas –> recurrent peptic ulceration
- Also adrenal and thyroid.
4
Q
What gene is mutated in MEN type 1?
A
MEN1 gene
5
Q
Where do tumours occur in MEN type IIa?
(Think 2 P’s)
A
- Medullary thyroid cancer (70%)
- Parathyroid –> Hypercalcaemia
- Phaeochromocytoma
6
Q
How does MEN type IIb present?
(Think 1P)
A
- Medullary thyroid cancer
- Phaeochromocytoma
- Marfanoid body habitus
- Neuromas
7
Q
What oncogene is mutated in MEN type IIa and MEN type IIb?
A
RET oncogene