Carcinoid Syndrome

INSTRUCTION

Look at this patient who was told by her GP that she has a 'nervous bowel'.

SALIENT FEATURES

History

· Confirm a history of chronic intermittent diarrhoea.

· Flushing attacks, which may be associated with increased lacrimation and

periorbital oedema. Flushing may be provoked by eating, exertion, excitement or

ethanol.

· Wheeze (due to bronchoconstriction during flushing attacks).

Examination

· Flushed face ('fire-engine' face).

· Telangiectasia.

Proceed as follows:

· Listen to the chest for wheeze (bronchial carcinoid).

· Listen to the heart (right-sided murmurs in intestinal, gastric, hepatic and ovarian

carcinoid, left-sided murmurs in bronchial carcinoid).

· Look for hepatomegaly (nodular and firm due to metastases, may be pulsatile due

to tricuspid regurgitation).

DIAGNOSIS

This patient has facial flushing and tricuspid regurgitation (lesion) due to carcinoid

syndrome (aetiology) and is in cardiac failure (functional status).

Classic reference: Am J Med 1956; 20- 520-32.

ADVANCED-LEVEL QUESTIONS

What are the cardiac lesions seen in metastatic carcinoid from the liver?

* Right-sided valvular lesions, including tricuspid stenosis or regurgitation, and

pulmonary stenosis or regurgitation. (Note: bronchial carcinoids metastasize to the

left side of the heart.)

· Endocardial fibrosis.

How is the diagnosis confirmed?

Raised urinary levels of 5-HIAA (hydroxyindoleacetic acid).

How are these tumours treated?

· Emergency treatment includes prednisolone.

. Severe diarrhoea: hydration, diphenoxylate with atropine, cyproheptadine or

methysergide.

. Octreotide (a somatostatin analogue) is associated with a significant reduction in

5-HIAA concentration.

* Surgery is useful for localized carcinoid.

· Chemotherapy in advanced disease (fluorouracil, streptozocin, dacarbazine).

· Interferon-c~ may be useful in those who do not respond to surgery and octreotide

treatment (Digestion 1994; 55 (suppl 3): 64-9).