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Flashcards in ENDOCRINE TUMOURS Deck (14)
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1
Q

How is MEN (multiple endocrine neoplasia) syndrome inherited?

A

Autosomal dominant

2
Q

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

A

They can be either

3
Q

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

A

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

4
Q

What is Werner’s syndrome?

A

MEN-I syndrome (multiple endocrine neoplasia)

5
Q

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

A

Zollinger-Ellison syndrome

6
Q

What is Sipple’s syndrome?

A

MEN-IIa (multiple endocrine neoplasia) syndrome

7
Q

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

A

Phaeochromocytoma (often bilateral)

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

8
Q

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

A

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

9
Q

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

A

Squamous cell carcinoma of the lung

Breast carcinoma

10
Q

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

A

Small cell carcinoma of the bronchus

Intestinal tumours

11
Q

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

A

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

12
Q

What are carcinoid tumours?

A

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.

13
Q

What is carcinoid syndrome?

A

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

14
Q

What are the clinical features of carcinoid syndrome?

A

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)