Tumours derived from neuro-endocrine or chromaffin cells. These may occur in isolation or as part of MEN1 or neurofibromatosis. The diagnosis carcinoid syndrome refers to the association of diarrhoea and flushing associated with peptide secretion from these tumours.
To diagnose neuro-endocrine tumours a detailed and full general examination is mandatory in the initial assessment of all patients.
Volume status should be carefully assessed in patients with diarrhoea.
Blood pressure may be low in patients with dehydration, dehydration or anterior pituitary failure and elevated in patients with phaeochromocytoma.
Corneal calcification may be apparent with hypercalcaemia.
Palpable lymph nodes are suggestive of metastatic disease.
Thymic hyperplasia associated with MEN1 is rarely palpable.
Carcinoid syndrome may cause right heart valve thickening and lead to congestive cardiac failure.
Bronchial carcinoids may also lead to signs in the chest.
Examine for pancreatic or bowel associated masses and hepatomegaly.
Seeking evidence of urogenital involvement, or renal disease due to hypercalcaemia.
Fixed or transient flushing is obvious as deep red or purple colouration to the skin particularly affecting the face and neck. Deep pigmentation may occur with ectopic ACTH secretion.
Glucagonoma may be associated with unusual skin changes in areas exposed to friction: necrolytic migratory erythema.
Facial angiofibromas, collagenomas and lipomas may be associated with MEN1.
Fine wrinkling of the skin occurs in hypopituitarism.
Pallor may also occur with anaemia of any cause.
Thin skin, multiple bruises, pigmented striae and hirsuitism may all occur with adrenal tumours or pituitary Cushing’s.
Doughy pallid 'myxoedematous' skin may be seen with loss of thyroid function. Doughy skin with soft tissue swelling also occurs in growth hormone excess.
The patient should be examined for signs of pituitary disease particularly in those with MEN1.