Multiple Endocrine Neoplasia Flashcards
1
Q
What is MEN? [2]
A
Autosomal dominant functioning hormone producing tumours in multiple organs
2
Q
How does MEN 1 occur? [3]
A
Mutation in the MEN 1 TSG
- > Defective menin protein production
- > Irregular cell growth
3
Q
What are the features of MEN 1? [4] PPPD
A
- Parathyroid Hyperplasia/Adenoma (hypercalcemia)
- Pancreatic tumours eg insulinoma (hypoglycaemia)
- Pituitary adenomas (mainly prolactinoma)
- Duodenal endocrine tumours (hypoglycaemia & ulcers)
4
Q
What cause MEN 2?
A
Mutations in RET proto-oncogenes
5
Q
What are the features of basic MEN 2? [2]
A
Medullary Carcinoma of thyroid Pheochromocytoma
6
Q
What are the features of the MEN 2A variant? [2]
A
MEN 2 triad
+ Parathyroid hyperplasia
7
Q
What are the features of the MEN 2B variant? [2]
A
MEN 2
+ Neuromas of skin, mucous membrane and skeletal abnormalities (Marfanoid body habitués)