MEN

Question 1

What is MEN

Answer 1

Functioning hormone producing tumours in multiple organcs

Question 2

Inhertiance

Answer 2

Autosomal dominant

Question 3

Examples

Answer 3

MEN1
MEN2
NF1
NF2
Von Hipple-Lindau
Peutz JAger

Question 4

MEN1

Answer 4

Parathyroid hyperplasia/adenoma - hypercalcaemia
Pnacreas endocrine tumours - grastrinoma or insulinoma
Pituitary prolacinoma/GH secreting tumour

Question 5

MEN2a

Answer 5

Thyroid - medullary thyroid carcinoma
Phaeochromocytoma
Parathyroid hyperplasia

Question 6

MEN2b

Answer 6

Mucosal neuromas
Marfanoid appearance
No hyperparathyroidism

Question 7

NF1

Answer 7

Cafe-au-lait spots - >6
Freckling
Dermal neurofibromas - small nodules which appear at pubertyu
Nodular neurofibromas - from nerve traunks
Lisch nodules in iris

Cx:
Learning disability
Nerve root compression
Malignancy - optic glioma

Mx:
MDT - genetics, neurologist, surgeons, PT
Excision if troublesome
Genetic counselling

Question 8

NF2

Answer 8

Bilateral acoustic neuroma (vestibular schwannoma)
SNHL by 20 y/o
Tinnitus and vertigo

Mx:
MRI brain

Question 9

Carcinoid syndrome

Answer 9

Tumours of neural crest cells - produce 5HT
Appendix
Ileum
Rectum

Tumours may secrete hormones - insulin, glucagon, ACTH, parathyroid, thyroid

Carcinoid syndrome implies hepatic involvement

CF
Bronchoconstriction
Flushing of upper body
Diarrhoea
CCF due to tricuspid incompetence and pulmonary stenosis

Ix:
Raised 5-HT in urine

Mx:
Ocrtreotide - blocks release of tumour mediators
Loperamide for diarrhoea
Resection of tumour

Question 10

Peutz Jager

Answer 10

Dark freckles on lips, mucosa, palms, soles
Multiple GI hamartomas causing obstruction, intussuseption or bleeds
Increased risk of GI cancer - colonoscopy and OGD every 3 years