Abstract

Neuroendocrine tumours are a heterogeneous group of neoplasms deriving from the neuroendocrine cell system, spread throughout the body. The most common localisation of the tumours is in the gastrointestinal tract including the pancreas, but also in the lung and thymus as well as in some endocrine organs. The tumours are considered to be rare, but recent data indicate an increased incidence and prevalence over the last two decades. The estimated incidence today is 4−5/100,000 per year and a prevalence of 35/100,000. A majority of patients with neuroendocrine tumours present metastatic disease (>60%) at the time of diagnosis. However, many tumours run an indolent course despite wide-spread disease and, therefore, surgery, peptide receptor radio therapy as well as medical treatment can be performed with long term control of the disease. The quality of life and also survival for most subtypes of neuroendocrine tumours have been significantly improved during the last decade. The precise genetic background for these diseases has not yet been defined, but some of the inherited forms of neuroendocrine tumours, such as multiple endocrine neoplasia type 1 (MEN1), endocrine neoplasia type 2 (MEN2) and von Hippel Lindau (vHL) are well characterised in terms of molecular genetics. The current symposium is presenting the most recent data in multiple endocrine neoplasia type 2 (MEN2). It is also providing a comprehensive overview about lung neuroendocrine tumours, which sometimes present a diagnostic and therapeutic challenge. To be able to make an accurate diagnosis and staging of neuroendocrine tumours, radio pharmaceutical investigation, such as somatostatin receptor scintigraphy, has been around for more than a decade. In this symposium, we will focus on the most recent development in peptide receptor radio treatment (PRRT) which has been introduced recently. Finally, surgery is one of the cornerstones in the management of neuroendocrine tumours and has been developed quite extensively during the last decade. The role of surgery in neuroendocrine tumours will be covered in this symposium.

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