Neuroendocrine tumours represent a group of very rare neoplasms, characterised by a high degree of histological and clinical heterogeneity. They can be silent (even producing some hormones) or clinically evident as a result of local growth and/or hormonal production. The therapeutic plan depends on a profound knowledge of the disease and the patient. It should be based on the following points: 1) histological diagnosis, including a proliferation index; 2) evaluation of the growth rate, by means of a radiological reassessment after one or two months without any anti-tumour therapy; 3) appropriate staging, using a total-body somatostatin-receptor scintigraphy, and computed tomography or magnetic resonance; 4) biochemical picture, including plasma chromogranin-A and possible specific hormones; 5) clinical evaluation, particularly evaluating the possible presence of a related syndrome; and, finally, 6) genetic evaluation, in order to verify the presence of a inherited disease. Therefore, the diagnostic and therapeutic approach should be multidisciplinary. The teem should include several experts, including an oncologist, radiologist, surgeon, nuclear medicine specialist, pathologist, clinical pathologist, gastroenterologist, endocrinologist and radiotherapist, who should discuss all of the cases and define therapeutic strategies rather than single therapy indications. Most patients have a slow-growing disease with a long-term survival even in presence of distant metastases. However, in a minority of cases the tumour is rapidly proliferative and prognosis is very poor. Somatostatin analogs should be used in functioning diseases and progressive somatostatin-receptor positive diseases. Chemotherapy is considered in rapidly progressive tumours. In patients with an indolent metastatic neuroendocrine tumour a cyto-reductive approach could be performed, including a debulking with hepatic chemoembolisation and/or surgery for primary tumours or metastases. In diseases highly expressing somatostatin receptors a targeted radiotherapy with 90Y-DOTATOC or 177Lu-DOTATATE can be considered.
|Translated title of the contribution||Neuroendocrine tumors|
|Number of pages||7|
|Publication status||Published - May 2007|
ASJC Scopus subject areas
- Internal Medicine