The p53 gene mapped on chromosome 17p is an important negative regulator of normal cell growth and division (Finlay et al., 1989;Chen et al., 1990;Diller et al., 1990;Isaacs et at., 1991). Alteration or inactivation of p53 by mutation can allow a cell to escape from normal into uncontrolled growth leading to cancer development. p53 mutations are a very common genetic change in a variety of human tumours Takahashi et al., 1989;Nigro et al., 1989;Bartek et al., 1990a;Marks et al., 1991;Tamura et al., 1991). The majority of the mutations alter the conformation of the nuclear protein product, encoded by the p53 gene (Gannon et al., 1990;Bartek et al., 1990b; Rodrigues et al., 1990). In normal cells and tissues the p53 protein has a very short half-life (Oren et al., 1981) and attains such a low level that it is not detectable histologically (Gannon et al., 1990). The mutant forms have an extended half-life (Finlay et al., 1988) and being overexpressed are readily detected by immunohistochemistry. Elevated p53 levels have been described in different human neoplasms (Crawford et al., 1984;Cattoretti et al., 1988;Bartek et al., 1990a;Iggo et al., 1990;Davidoff et al., 1991a;Marks et al., 1991
An increased interest in gastro-entero-pancreatic neuroendocrine neoplasms (GEP NENs) has recently been observed. These are rare neoplasms and their detection in recent years has improved. Over 50% of GEP NENs are carcinoids, and they are usually found incidentally during surgery in the small intestine and appendix and at diagnosis in distant metastases, mainly to the liver. There is a need for co-operation between specialists in various disciplines of medicine in order to work out the diagnostic and therapeutic guidelines. In this publication, we present general recommendations of the Polish Network of Neuroendocrine Tumours for the management of patients with GEP NENs, developed at the Consensus Conference which took place in Kamień Śląski in April 2013. Members of the guidelines working groups were assigned sections of the 2008 guidance to update. In the subsequent parts of this publication, we present the rules of diagnostic and therapeutic management of: -neuroendocrine neoplasms of the stomach and duodenum (including gastrinoma); -pancreatic neuroendocrine neoplasms; -neuroendocrine neoplasms of the small intestine and the appendix; -colorectal neuroendocrine neoplasms. The proposed recommendations by Polish and foreign experts representing different fields of medicine (endocrinology, gastroenterology, surgery, oncology, nuclear medicine and pathology) will be helpful in the diagnosis and treatment of GEP NENs patients.
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