Neuroendocrine tumours may be associated with familial syndromes. At least eight inherited syndromes predisposing to endocrine neoplasia have been identified. Two of these are considered to be major factors predisposing to benign and malignant endocrine tumours, designated multiple endocrine neoplasia type 1 and type 2 (MEN1 and MEN2). Five other autosomal dominant diseases show more heterogene...

Neuroendocrine neoplasms are defined as epithelial neoplasms with predominant neuroendocrine differentiation. They can arise in almost every organ of the body although they are most commonly found in the gastrointestinal tract and respiratory system. Nasal cavity and paranasal sinuses are a rare site for neuroendocrine carcinoma. In contrast to the other regions, neuroendocrine tumours of the s...

Neuroendocrine tumours of the pancreas can have a spectrum of behaviour from relatively benign to aggressive. Resection can result in cure although metastatic disease is described. We present an unusual case of an apparent local recurrence of previously resected neuroendocrine tumour in a young man who had undergone distal pancreatectomy. Pathological analysis demonstrated focal post-surgical p...

BACKGROUND/AIMS To identify potential preoperative prognostic factors in resected pancreatic and periampullary neuroendocrine tumours. METHODS Clinico-pathological data for 54 consecutive patients with pancreatic or periampullary neuroendocrine tumours referred to our institution over a 10-year period were identified from a prospective database. RESULTS 34 patients underwent pancreatic rese...

Aproximately 5-10% of neuroendocrine tumours (NETs) of the gastroenteropancreatic system (GEP) have an hereditary background. The known hereditary syndromes include: multiple endocrine neoplasia type 1 (MEN 1), von Hippel Lindau disease (VHL), neurofibromatosis type 1 (NF 1) and tuberous sclerosis complex (TSC). This review discusses for each of these syndromes the: genes involved and specifics...

This paper presents the updated Polish Neuroendocrine Tumour Network expert panel recommendations on the management of neuroendocrine neoplasms (NENs) of the stomach and duodenum, including gastrinoma. The recommendations discuss the epidemiology, pathogenesis and clinical presentation of these tumours as well as their diagnosis, including biochemical, histopathological and localisation diagnos...

Aim To provide an educational update on structural imaging appearances of neuroendocrine tumours (NET), in the age of molecular imaging. PET/CT with Ga-68 DOTA-TATE and F-18 fluorodeoxyglucose (FDG) is providing new understanding of neuroendocrine tumours including patterns and heterogeneity of disease. This is also providing new insights of structural imaging findings including CT and MRI. It ...

Small cell carcinoma of the endometrium is extremely rare. The clinical behaviour of this tumour is usually very aggressive. The histopathological distinction of endometrial small cell neuroendocrine carcinoma from mesodermal mixed tumours has important prognostic and therapeutic implications. The case of a 62-year-old white female suffering from postmenopausal bleeding is presented. Macroscopi...

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...

Secreting Gastro-enteropancreatic Neuroendocrine Tumours and Biomarkers 1 2 Wieke H.M. Verbeek 1* , Catharina M. Korse 2* , Margot E.T. Tesselaar 3* 3 Departments of 1 Gastroenterology, 2 Clinical Chemistry and 3 Medical Oncology 4 The Netherlands Cancer Institute – Antoni van Leeuwenhoek, Amsterdam 5 The Netherlands 6 * Equal contribution 7 8 Corresponding author: 9 Dr. M.E.T. Tesselaar, Plesm...