Carcinoid heart disease: an update.

BACKGROUND: Carcinoid tumours are a poorly defined collection of lesions, histopathologically indistinguishable from gastroentero-pancreatic neuroendocrine tumours. In this report, we discuss epidemiology and survival, clinical presentation, carcinoid valvular heart disease (CVHD), histopathological considerations and treatment options. METHODS: Review and update of the literature. RESULTS: The term carcinoid suggests a disease entity, but with increasing knowledge it becomes progressively confusing. To avoid further confusion, it is advisable to define these tumours using differentiation, stage, primary site, known tumour products and an associated clinical syndrome. Incidence varies between 0.8 and 1.9/100,000 population. About 20% present with metastases, with a 5-year survival varying between 15% and 35%. Metastatic disease frequently accompanies the carcinoid syndrome (flushing, diarrhoea, wheezing and CVHD). CVHD incidence is about 50%, and seems unrelated to disease duration and tumour mass. An aetiological relation of CVHD with urinary 5-HIAA remains to be confirmed. Resection is the only curative option. Surgery can also offer prolonged palliation and is needed to restore bowel transit in obstructive/ischaemic bowel problems. Adequate palliation of hormone-related symptoms can also be achieved by somatostatin analogues, meta-iodo-benzyl-guanidine preparations and interferon-alpha formulations, all with a 70% response rate. Embolization of liver metastases has led to objective responses in about 50% of patients, but is accompanied by significant side effects. CONCLUSIONS: Most patients are cured by surgery. Symptom relief is the main target in metastatic disease and can be achieved by a range of equally potent biologically active medications, debulking surgery and hepatic embolization.