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Carcinoid Syndrome

Title               

Ñ    Carcinoid Syndrome

Definition

Ñ    Hormonal manifestations of carcinoid tumours

Pathogenesis

Ñ    Metastases from intestinal or bronchial carcinoid tumours

Ñ    Other sites rarely

Ñ    Tumour arises from neuroendocrine cells

Ñ    Metastasises to liver

Ñ    Produces amines and polypeptides which cause clinical features

Ñ    Most significantly:

Serotonin

Clinical Features

History

Age

Ñ    50-70 yrs

Sex

Ñ    Males = Females

IS

Ñ    Flushing:

·          Spontaneous

Or

·          Induced by excitement, exercise, some types of food, alcohol

CVS

Ñ    Right heart failure

RS

Ñ    Asthma

Ñ    Cough, haemoptysis:

Primary tumour

GIT

Ñ    Diarrhoea:

Episodic

Occurring after meals

Ñ    Abdominal pain:

Obstruction due to tumour or desmoplastic reaction caused by it

Examination

E&M

Ñ    Pellagra:

·          Dementia, diarrhoea, dermatitis:

Due to diversion of dietary tryptophan to serotonin

IS

Ñ    Flushing

Ñ    Telangiectasia

CVS

Ñ    Tricuspid incompetence or stenosis

Ñ    Pulmonary stenosis or incompetence:

Rarely

RS

Ñ    Wheezing

GIT

Ñ    Abdominal mass

Ñ    Hepatomegaly

Investigations

Fluids

Urine

Biochemistry

Ñ    5 hydroxy indole acetic acid (5 HIAA):

Increased

Imaging

Ultrasound

Ñ    Liver metastases

CT Scan

Ñ    Investigation of choice:

Non-contrast

MRI

Ñ    Increasingly used

Nuclear Medicine

Ñ    Octreotide scan

Ñ    Positron emission tomography (PET):

Experience limited

Endoscopy

Gastrointestinal

Ñ    May sometimes show up primary tumour

Bronchoscopy

Ñ    May be helpful in bronchial disease

Surgery

Laparoscopy

Ñ    May be useful for localising lesions and obtaining tissue

Tissue Diagnosis

Biopsy

Ñ    Uniform small cells arranged as islands separated by a fibrous stroma

Ñ    Scant pink cytoplasm, finely granulated, stippled, small round nuclei and small nucleoli.

Management

Control

Drugs

Ñ    Long acting octreotide:

Inhibits release of hormones, may inhibit tumour growth

Ñ    Interferon alpha:

Transient benefit, side effects

Ñ    Streptozotocin, cisplatin, etoposide, and doxorubicin:

Alone or in combination has shown some success

Surgery

Ñ    Curative resection or debulking

Symptom Relief

Radiotherapy

Ñ    Palliation of bone metastases

Prognosis

Ñ    Good compared to other malignancies

 

 

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