Carcinoid syndrome

Published on 02/04/2015 by admin

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Last modified 22/04/2025

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147 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 (caused by 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 from metastases, may be pulsatile from tricuspid regurgitation).

Diagnosis

This patient has facial flushing and tricuspid regurgitation (lesion) caused by carcinoid syndrome (aetiology) and is in cardiac failure (functional status).

Remember

• Carcinoid tumours, now referred to as neuroendocrine tumours (NETs). NETs include a wide spectrum of neoplasms and clinical behaviours depending on their site of origin, hormonal production, and differentiation.

• Be prepared to discuss carcinoid biochemical pathways.

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.

Remember: Serotonin is related to the progression of carcinoid heart disease, and the risk of progressive heart disease is higher in patients who receive chemotherapy than in those who do not (N Engl J Med 2003;348:1005–15).

What are the types of gastric carcinoid?

There are three types:

Type I is associated with chronic atrophic gastritis.

Type II develops in patients with combined multiple endocrine neoplasia type 1 and the Zollinger–Ellison syndrome. (The multiple endocrine neoplasia type 1 gene locus may be involved in type II gastric carcinoid tumours.)

Type III is sporadic.

Note: Hypergastrinaemia has an important role in the development of types I and II.

How is the diagnosis confirmed?

• Raised urinary levels of 5-HIAA (hydroxyindoleacetic acid; Fig. 147.1)

• Plasma and platelet serotonin levels.

image

Fig. 147.1 Typical biochemical carcinoid pathway.

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

• Hepatic artery embolization

• Chemotherapy in advanced disease (fluorouracil, streptozocin, dacarbazine)

• Interferon-α may be useful in those who do not respond to surgery and octreotide treatment (Digestion 1994;55(suppl 3):64–9).

Further reading

Sjoerdsma A, Weissbach H, Udenfriend S. A clinical, physiologic and biochemical study of patients with malignant carcinoid (argentaffinoma). Am J Med. 1956;20:520-532.

Carcinoid was described in 1888 by Lubarsch, the term Karznoid, or carcinoma-like, was introduced by Oberndorfer in 1907 when he described a midgut tumour that was morphologically distinct and less aggressive in behaviour than intestinal adenocarcinoma

In 1914, Gosset and Mason suggested that carcinoid tumours arose from the enterochromaffin cells of the GI tract.

Erspamer succeeded in demonstrating serotonin (5-HT) in the enterochromaffin cells and in 1953 Lembeck was able to extract 5-HT from a carcinoid tumour.

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