Multiple Endocrine Neoplasia

Question 1

What does this stand for?

Answer 1

Multiple Endocrine Neoplasia

Question 2

What is it?

It is a genetic disease. Is autosomal dominant or autosomal recessive?

Answer 2

They are functioning hormone-producing tumours in multiple organs.

Autosomal dominant

Question 3

MEN-1:

What is another name for this?

What 3 places are tumours found - 3P’s

Answer 3

Wermer’s syndrome

Parathyroid gland

Pituitary gland

Pancreas (it is still a gland)

Question 4

MEN-1:

What does parathyroid adenoma or hyperplasia lead to?

What does a pituitary prolactinoma or GH secreting tumour lead to?

Pancreas endocrine tumours - what hormones could they possibly produce in excess?

Answer 4

Hyperparathyroidism

Acromegaly

Insulin - insulinoma
Gastrin - Gastrinoma-
Glucagon - glucogonomas

Question 5

MEN 2α:

What 3 places are tumours found?

Answer 5

Parathyroid gland

Adrenal gland - phaeochromocytoma

Thyroid gland - medullary thyroid cancer

Question 6

Men 2b:

What 2 places are tumours found?

They are described as having a marfanoid appearance. What does this mean?

What are mucosal neuromas?

Answer 6

Adrenal gland - phaeochromocytoma

Thyroid gland - medullary thyroid cancer

Marfanoid (or Marfanoid habitus) is a constellation of symptoms resembling those of Marfan syndrome, including long limbs, with an arm span that is at least 1.03 of the height of the individual, and a crowded oral maxilla, sometimes with a high arch in the palate, arachnodactyly, and hyperlaxity.

Yellow-white nodules on lips and tongue