ENDOCRINE TUMOURS

Question 1

How is MEN (multiple endocrine neoplasia) syndrome inherited?

Answer 1

Autosomal dominant

Question 2

Are tumours associated with MEN (multiple endocrine neoplasia) syndrome benign or malignant?

Answer 2

They can be either

Question 3

What are the most common tumours associated with MEN-I (multiple endocrine neoplasia type 1) syndrome?

Answer 3

Parathyroid hyperplasia
Pancreatic islet cell tumours (insuloma or glucagonoma)
Duodenal tumours
Pituitary adenoma (GH, prolactin or ACTH)

Question 4

What is Werner’s syndrome?

Answer 4

MEN-I syndrome (multiple endocrine neoplasia)

Question 5

In patients with MEN-I syndrome, what is the rare syndrome that can result from a duodenal tumour?

Answer 5

Zollinger-Ellison syndrome

Question 6

What is Sipple’s syndrome?

Answer 6

MEN-IIa (multiple endocrine neoplasia) syndrome

Question 7

What are the most common tumours associated with MEN-IIa (multiple endocrine neoplasia type 2a) syndrome?

Answer 7

Phaeochromocytoma (often bilateral)

Medullary cell carcinoma of the thyroid (presents often before the age of 30)

Question 8

What are the most common tumours associated with MEN-IIb (multiple endocrine neoplasia type 2b) syndrome?

Answer 8

Phaeochromocytoma
Medullary cell carcinoma of the thyroid (presents often before the age of 5)
Neuroma
Ganglioneuromas

Question 9

What tumours have been known to cause hypercalcaemia due to ectopic release of PTH or PTH-like peptide?

Answer 9

Squamous cell carcinoma of the lung

Breast carcinoma

Question 10

What tumours have been known to cause hyponatraemia due to ectopic release of ADH?

Answer 10

Small cell carcinoma of the bronchus

Intestinal tumours

Question 11

What tumours have been known to cause hypokalaemia due to ectopic release of ACTH?

Answer 11

Small cell carcinoma of the bronchus
Medullary carcinoma of the thyroid (MTH)
Thymic carcinoma
Islet cell tumours

Question 12

What are carcinoid tumours?

Answer 12

Tumours of the APUD cells of the intestine, most commonly affecting the appendix, ileum and rectum. They can secrete a variety of hormones including insulin, glucagon, ACTH, thyroid and PTH. Can appear as part of MEN-I syndrome.

Question 13

What is carcinoid syndrome?

Answer 13

Occurs in around 5% of patients with carcinoid tumours (tumours of APUD cells) and indicates liver metastases involvement.

Question 14

What are the clinical features of carcinoid syndrome?

Answer 14

Flushing, especially of face and neck (most likely as a result of bradykinins)
Diarrhoea
Abdominal pain
Cardiac complications (due to serotonin induced pulmonary fibrosis and tricuspid incompetence)