Endocrine tumours of the pancreas

• Published in: Baillière's best practice & research. Clinical gastroenterology 2005-10, Vol.19 (5), p.753-781
• Main Authors: Öberg, Kjell, Eriksson, Barbro
• Format: Article
• Language: English
• Subjects: biochemistry; Biology; Biopsy, Needle; Carcinoma, Neuroendocrine - drug therapy; Carcinoma, Neuroendocrine - epidemiology; Carcinoma, Neuroendocrine - genetics; Carcinoma, Neuroendocrine - pathology; Comparative Study; Cytotoxicity; endocrine pancreatic tumours; Female; Gastrinoma - drug therapy; Gastrinoma - epidemiology; Gastrinoma - pathology; Gastrinoma/drug therapy/epidemiology/pathology; Glucagonoma - drug therapy; Glucagonoma - epidemiology; Glucagonoma - pathology; Glucagonoma/drug therapy/epidemiology/pathology; Growth hormones; histopathology; Humans; Immunohistochemistry; Incidence; Insulinoma - drug therapy; Insulinoma - epidemiology; Insulinoma - pathology; Insulinoma/drug therapy/epidemiology/pathology; localization; Male; medical treatment; Molecular Biology; molecular genetics; Neoplasm Staging; Pancreas; Pancreatic Neoplasms - drug therapy; Pancreatic Neoplasms - epidemiology; Pancreatic Neoplasms - genetics; Pancreatic Neoplasms - pathology; Pancreatic Neoplasms/drug therapy/epidemiology/genetics/pathology; Polypeptides; Prognosis; Risk Assessment; Somatostatinoma - drug therapy; Somatostatinoma - epidemiology; Somatostatinoma - pathology; Somatostatinoma/drug therapy/epidemiology/pathology; Stem cells; Survival Rate; Tomography; Treatment Outcome; Ultrasonic imaging; Zollinger-Ellison Syndrome - drug therapy; Zollinger-Ellison Syndrome - epidemiology; Zollinger-Ellison Syndrome - pathology; Zollinger-Ellison Syndrome/drug therapy/epidemiology/pathology
• ISSN: 1521-6918; 1532-1916
• DOI: 10.1016/j.bpg.2005.06.002

Endocrine pancreatic tumours (EPTs) are uncommon tumours occurring in approximately 1 in 100 000 of the population, representing 1–2% of all pancreatic neoplasms. Some of the tumours may be part of multiple endocrine neoplasia type one (MEN-1) syndrome or von Hippel–Lindau (vHL) disease. EPTs are classified as functioning or non-functioning tumours on the basis of their clinical manifestation. The biochemical diagnosis of EPT is based on hormones and amines released. Besides specific markers such as insulin, there are also general tumour markers such as chromogranin A, which is the most valuable marker and has been reported to be increased in plasma in 50–80% of patients with EPTs and correlates with tumour burden. The location of endocrine tumours of the pancreas includes different techniques, from endoscopic investigations to scintigraphy (e.g. somatostatin receptor scintigraphy) and positron emission tomography. The medical treatment of endocrine pancreatic tumours consists of chemotherapy, somatostatin analogues and α-interferon. None of these can cure a patient with malignant disease. In future, therapy will be custom-made and based on current knowledge of tumour biology and molecular genetics.