Multiple endocrine neoplasia Flashcards
1
Q
Inheritance?
A
Autosomal dominant disorder
2
Q
How many types of MEN are there?
A
3:
MEN type 1
MEN type 2a
MEN type 2b
3
Q
What is affected in MEN type 1?
A
3 P’s
- Parathyroid (95%): hyperparathyroidism due to parathyroid hyperplasia
- Pituitary (70%)
- Pancreas (50%): e.g. insulinoma, gastrinoma (leading to recurrent peptic ulceration)
Also: adrenal and thyroid
4
Q
What gene is responsible for MEN type 1?
A
MEN1 gene
5
Q
How does MEN type 1 most commonly present?
A
With hypercalcaemia
6
Q
What symptoms are in MEN type 2a?
A
Medullary thyroid cancer (70%)
2 P’s
- Parathyroid (60%)
- Phaeochromocytoma
7
Q
What gene is responsible for MEN type 2a?
A
RET oncogene
8
Q
What gene is responsible for MEN type 2b?
A
RET oncogene
9
Q
What occurs in MEN type 2b?
A
Medullary thyroid cancer
1 P
Phaeochromocytoma
Marfanoid body habitus
Neuromas