Neuroendocrine tumours (NETs) have an age-adjusted incidence of 1.2-2.1 per 100,000. We have studied 36 such patients with NETs who are being followed up in our multidisciplinary clinic. Of these 36 patients, there are 17 males (median age 71, range 55-88) and 19 females (median age 66, range 36-79). The presenting features were highly variable; only three patients (8.3 percent) presented with the carcinoid syndrome, eight patients (22.2 percent) presented with small bowel obstruction. Other presenting features varied depending on the site e.g. effects of liver metastases, ovarian cysts and radiological evidence of bronchial lesions. Seventeen patients (47 percent) had small bowel primaries, 3 (8.3 percent) had bronchial primaries, 2 (5.5 percent) had large bowel primaries, one (2.7 percent) patient had an ovarian primary, one (2.7 percent) had a gastric primary and in the remaining 12 patients (33.3 percent), the primary was unknown. Twenty five patients (69.4 percent) had the carcinoid syndrome, characterised by flushing, wheezing, diarrhoea or pruritus. Of the sixteen patients who had echocardiograms, only two had evidence of carcinoid heart disease. Twenty six patients (72.2 percent) are currently on a somatostatin analogue. Two patients have been referred for chemotherapy, one for hepatic artery embolisation, and 3 have been referred for Iodine 131-labelled meta-iodobenzylguanidine (MIBG) therapy. The current clinical strategy is to locate the site of the primary lesion where possible, in addition to investigating the extent of spread of the tumour, evaluating urinary 5-HIAA levels, serum chromogranins (A and B) and assessing MIBG uptake status to select patients for MIBG labelled therapy. The patients who are MIBG 'negative' are offered the opportunity to participate in a chemotherapy trial. The appropriate patients are commenced on somatostatin analogues. NETs require a multimodal and multidisciplinary approach to provide high quality patient care and to strengthen the evidence base for management.
22 - 24 Mar 2004
British Endocrine Societies