Paediatric pancreatic neuroendocrine tumours in von Hippel–Lindau disease

Somatostatin receptors in gastroentero-pancreatic neuroendocrine tumours.

Five somatostatin receptor (sst) subtype genes, sst(1), sst(2), sst(3), sst(4) and sst(5), have been cloned and characterised. The five sst subtypes all bind natural somatostatin-14 and somatostatin-28 with high affinity. Endocrine pancreatic and endocrine digestive tract tumours also express multiple sst subtypes, but sst(2) predominance is generally found. However, there is considerable varia...

Whole-genome landscape of pancreatic neuroendocrine tumours

The diagnosis of pancreatic neuroendocrine tumours (PanNETs) is increasing owing to more sensitive detection methods, and this increase is creating challenges for clinical management. We performed whole-genome sequencing of 102 primary PanNETs and defined the genomic events that characterize their pathogenesis. Here we describe the mutational signatures they harbour, including a deficiency in G...

Acute pancreatitis secondary to pancreatic neuroendocrine tumours.

CONTEXT Pancreatic neoplasms are an uncommon aetiology of acute pancreatitis. Pancreatic neuroendocrine tumours are a rare subgroup of pancreatic neoplasms. CASE REPORT We report on three patients having acute pancreatitis secondary to pancreatic neuroendocrine tumours, one of them with severe pancreatitis, and review the published cases up to now. Only 22 patients with acute pancreatitis sec...

Appendicular Neuroendocrine Tumours in Children: Unicentric Retrospective Study

Background: Neuroendocrine Neoplasms (NEN) represent 60% of all appendicular tumours. This type of cancer is predominantly benign.  In this study, appendicular NEN tumours in children were investigated. Materials and Methods: This retrospective study was conducted on 540 patients underwent emergency appendectomy for the treatment of clinically suspected appendicitis at the department of p...

Double Hormone Syndromes in Two Pancreatic Neuroendocrine Tumours