Somatostatin receptor expression in multiple endocrine neoplasia and in von Hippel-Lindau disease

Somatostatin receptors are expressed on the majority of neuroendocrine tumours. The presence of these receptors is clinically useful. First, long- term treatment with somatostatin analogues controls hormonal hypersecretion, which controls flushing attacks, watery diarrhoea, hypoglycaemia and electrolyte disorders in patients with carcinoids and islet cell tumours. Secondly, somatostatin receptor imaging is used to localize primary neuroendocrine tumours and to visualize the spread of the disease. Thirdly internalization of somatostatin receptors by primary neuroendocrine tumours opens the possibility of carrying out radio- and chemotherapy with somatostatin analogues coupled to beta-emitting radionuclides and chemotherapeutic drugs. The presence and role of somatostatin receptors on the tumours which occur in multiple endocrine neoplasia and von Hippel- Lindau disease are discussed.

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