Multiple Endocrine Neoplasia

Question 1

Define multiple endocrine neoplasia

Answer 1

ereditary tumour syndromes of variable neoplastic patterns and characterised by the development of multiple endocrine tumours.
Classification:
- MEN1/ Wermer’s Syndrome
- MEN2
> MEN2A/ Sipple’s Syndrome
> MEN2B
> Familial Medullary Thyroid Cancer: Family pattern of isolated medullary thyroid cancers with no other MEN2 manifestations.

MEN1 
• Pituitary Adenoma  
• Meningiomas 
• Thyroid Adenomas 
• Parathyroid Adenomas 
• Carcinoid Tumours 
• Gastrinomas 
• Pancreatic Endocrine Tumours 
- Gastrinoma 
- Insulinoma 
- Glucagonoma 
- Somatostatioma 
• Lipomas 
• Facial Angiofibromas 
• Skin tumours 

MEN2A
• Medullary Thyroid Cancer
• Phaeochromocytoma
• Malignant Parathyroid Adenoma with Hyperparathyroidism.
• Hirschprung’s Disease –congenial Megacolon.
• Cutaneous Lichen Amyloidosis.

MEN2B 
• Medullary Thyroid Cancer 
• Phaeochromocytoma 
• Marfanoid Body Habitus 
• Mucosal Intestinal Ganglioneuromatosis