Neuroendocrine gastrointestinal tumours
Open Access
- 1 July 1996
- journal article
- review article
- Published by Elsevier BV in Annals Of Oncology
- Vol. 7 (5), 453-463
- https://doi.org/10.1093/oxfordjournals.annonc.a010633
Abstract
Neuroendocrine gut and pancreatic tumours have provided a diagnostic and therapeutic challenge over the years. These rather slowly growing neoplasms have been assigned a good prognosis but when liver metastases are present the prognosis is not better than that of most other malignant tumours. Despite the development of improved diagnostic procedures many patients are still referred at a stage of the disease too late for surgical cure, at which time medical treatment is warranted. The diagnosis is based on histopathological diagnosis including silver stainings (Grimelius, Masson) and imrnunohistochemistry for chromogranin A and synaptophysin. Analysis of chromogranin A in the plasma is an important adjunct in the screening for various types of neuroendocrine gut and pancreatic tumours. About 80%–100% of patients with verified neuroendocrine gastrointestinal turnours have elevated circulating levels of this glycoprotein. Depending on clinical symptoms the chromogranin A analysis is supplemented by other peptide hormone analyses as well as urinary 5-HIAA for patients with midgut carcinoid tumours. In the past the localization procedures were based on CT, MRI and ultrasound investigations but in recent years somatostatin receptor scintigraphy (octreoscan) and endoscopic ultrasonography have significantly improved the diagnostic potential. Almost 80% of neuroendocrine gastrointestinal tumours present somatostatin receptor subtype 2 binding 111Indiumlabelled octreotide which can be used for staging of the disease, and which also indicates whether or not somatostatin analogues can be used in the treatment of these tumours. Surgery is still a cornerstone in the treatment of neuroendocrine gastrointestinal tumours, even if the patients are beyond cure. Debulking procedures and bypassing operations are important for improving clinical condition and facilitating impending medical treatment, and during the past decade a more aggressive surgical approach has emerged. The medical treatment is based on chemotherapy, and the use of somatostatin analogues and alpha-interferons. Chemotherapy, in particular the combination of streptozotocin with 5-FU or doxorubicin, is still first-line treatment for most endocrine pancreatic tumours, while somatostatin analogues and alphainterferons are considered first-line for classical midgut carcinoids. Chemotherapy and biotherapy can be combined in many patients, and changes from one medical treatment to another during the course of the disease is mandatory for control of the disease. It is important to realise that most patients with malignant tumours are not cured by medical treatment but that the disease can be controlled for extended periods of time. In the future it will be possible to individualize treatments on the basis of new information about such features of tumour biology as proliferation capacity, expression of adhesion molecules, and growth factors and their receptors.Keywords
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