P-0030 - Most prevalent pancreatic neuroendocrine tumors in everyday clinical practice
|Date||28 June 2014|
|Event||World GI 2014|
|Topics|| Neuroendocrine Cancers
|Citation||Annals of Oncology (2014) 25 (suppl_2): ii14-ii104. 10.1093/annonc/mdu165|
I. Souza, L. Vasques, S. Silva, M. de Oliveira, V. de Paiva
Pancreatic Neuroendocrine Tumors (NET) are epithelial neoplasias with predominant neuroendocrine differentiation. They are rare (annual incidence of less than one case over 100.000 people), equally affecting men and women between 30 and 60 years. They correspond to 3% of the pancreatic tumors and they are characterized by their ability of storing and secreting peptides and neuroamines. When compared to tumors of the exocrine pancreas, they present better prognosis and long-term survival rate. They affect mainly the tail of the pancreas (48% of cases), but it is possible that they sporadically occur out of the gland, or in association to a syndrome of endocrine hereditary neoplasia. They are classified as functional or non-functional (depending if they are associated or not to this syndrome), benign, malignant or indeterminable. Its diagnosis generally relies on medical imaging, once the localisation of the tumor is fundamental to determine its management, but the hormonal dosage of markers of endocrine production (such as cromogranina A) is often used. Surgical intervention is the only potentially curative treatment. Insulinome, gastrinome and glucagonome are the most prevalent types of pancreatic NET. Therefore a literature review on these tumors is proposed, with regards to their clinical, epidemiological and therapeutic aspects.
Researches through articles published from 2010 onwards, on Scielo, PubMed and UpToDate platforms.
Insulinomes make up 30 to 45% of all pancreatic NET, whose etiology and pathogenesis remain unknown. They are equally distributed all over the gland and the majority of them are benign, small and sporadic. Once they are developed from beta cells of pancreatic islets, patients present with persistent symptoms of hypoglycemia. Gastrinomes correspond to 20% of pancreatic NET. As the insulinomes, their etiology and pathogenesis remain unknown and they are preferably located on the “Triangle of Gastrinomes”, from which take part the head and the body of pancreas. The excess of gastrin that is secreted results in intense abdominal pain, secondary to gastric ulcers and subsequent reflux. Glucagonomes represent 10% of pancreatic NET and 75% of them are malignant. The symptoms vary from moderate glucose intolerance to weight gain, and the finding of a necrolytic migratory erythema is very characteristic. Within these three types of tumors, surgical resection is the elected treatment, either by open or laparoscopic view, and it must be considered in all patients who present with located tumor.
Despite their rarity, pancreatic NET require knowledge of physicians from different specialties, once they are manifested by common signs and symptoms in everyday clinical practice. There is increasing evidence that the prognosis has greatly improved in recent years, due to earlier diagnoses, more precise tumor localisation and definition of a more targeted surgical strategy.