MEN 1, 2a and 2b. Polyendrocrine disorders

Question 1

What is a MEN

Answer 1

Functional hormone producing tumours in multiple organs

Question 2

how are MENs inherited

Answer 2

Autosomal Dominant

Question 3

What organs are associated with MEN-1 (3)

Answer 3

PPP (50,70,95% frequency)
Pituitary Prolactinoma
Pancreas endocrine tumour – (Gastrin,insulin,somatostatin,VIP,Glucagonoma)
Parathyroid hyperplasia WITH increased Ca2+ in most

Question 4

Is MEN 1 a TSG or Oncogene

Answer 4

TSG (protein = menin)

Question 5

What are the decades of peak prevalence

Answer 5

30-50

Question 6

What are the MEN-2a associated tumours

Answer 6

TAP

Thyroid: medullary (100%)
Adrenal: phaechromocytoma (usually benign/bilateral)
Parathyroid hyperplasia WITHOUT increased Ca2+ (only 20%)

Question 7

What features does MEN-2b NOT share with 2a

Answer 7

No hyperparathyroidism

Question 8

What additional features does MEN-2b have on 2a (2)

Answer 8

Marfanoid features

Mucosal neuromas

Question 9

What are mucosal neuromas and where are they found

Answer 9

Bumps on lips/cheeks/tongue/glottis/visible corneal nerves

Question 10

Name the 4 main MENs (+1 more rarer)

Answer 10

MEN1
MEN2
Von Hippel Lindau
Neurofibromatosis
(+Peutz-Jeghers)

Question 11

What does Von hippel lindau predispose you to (4)

Answer 11

Bilateral renal cysts
retinal haemangioblastoma
Cerebellar haemangioblastoma
Phaechromocytoma

and therefore visual/cerebellar impairment signs