Neuroendocrine tumors of the gastro-entero-pancreatic system: the role of early diagnosis, genetic testing and preventive surgery.

Surgery is the only curative approach in neuroendocrine gastro-entero-pancreatic (GEP) tumors. As cure is highly dependent of tumor size, early diagnosis is a prerequisite for surgical success. Diagnosis of nonfunctioning tumors of the pancreas or midgut origin is due to symptoms related to the tumor burden, thus early diagnosis is mostly incidental. Functioning pancreatic tumors should be operated early in the course of the disease to provide cure. No genetic screening is available for sporadic GEP tumors. In patients with MEN-1 syndrome genetic screening is recommended to restrict the burden of clinical screening to those with positive test results. Due to the nature of the disease cure may not be achieved, however prevention of cancer may be obtained for one or two decades of life, especially in patients with gastrinoma or nonfunctioning tumors. Copyright 2002 S. Karger AG, Basel