A Rare Cause of Hyponatremia

Neuroendocrinology, 2009

Cancer Treatment Reviews, 1994

Pancreas, 2010

Summary Indroduction: Neuroendocrine tumors (NEN) of the gastro-entero-pancreatic tract (GEP) are a group in themselves very heterogeneous of tumors that are different for the site of localization in the digestive tract (foregut, midgut and hindgut), both in relation to the pathological aspects, functional activity and nosographic classification.

Endocrine-Related Cancer, 2016

Although recent epidemiological evidence indicates that the prevalence of non-functioning gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) is rising, a significant number of GEP-NETs still present with symptoms related to the secretion of biologically active substances leading to the development of distinct clinical syndromes. In the past, these syndromes were associated with substantial morbidity and mortality due to the lack of specific therapies; however, since the introduction of long-acting somatostatin analogues and medications such as proton pump inhibitors, their control has been greatly improved. As a result, nowadays, the main cause of morbidity and mortality in GEP-NETs is mostly directly related to tumour growth and the extent of metastatic disease. However, in some patients with functioning tumours and extensive disease, control of the secretory syndrome still remains problematic, necessitating the employment of several cytoreductive techniques, which may not ...

Neuroendocrinology, 2012

Endokrynologia Polska, 2022

This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially  Guidelines Beata Kos-Kudła et al. Guidelines persons/year, with the primary lesion most frequently found in the small intestine (37.4%). Since 2000, rectal NENs have been diagnosed more frequently than small intestine NENs [2, 6-8]. The incidence of GEP-NETs in the USA, based on SEER data, was 3.56/100,000 persons/year. In Europe, the incidence of GEP-NETs is also increasing-from 1.33 to 2.33/100,000 persons/year; however, these data come from different registries and are mainly retrospective. Higher incidence is observed among men (5.35/100,000 persons/year) compared to women (4.76/100,000 persons/year) [1, 5-7]. The vast majority of NENs are sporadic, well-differentiated tumours. However, GEP-NETs originating from the pancreas, duodenum, stomach, and much less frequently, from the thymus and lungs sometimes constitute an element of multiple endocrine neoplasia type 1 (MEN-1) syndrome. Pancreatic neuroendocrine tumours (PanNETs) may also be associated with von Hippel-Lindau (VHL) syndrome, tuberous sclerosis complex (TSC), and neurofibromatosis (NF). In these congenital diseases, NETs can be multifocal and occur 10-20 years earlier than in sporadic cases. The frequency of the hereditary causes (MEN-1, VHL) is estimated at about 5%. Genome studies revealed the presence of germline mutations in, e.g., MUTYH, CHEK2, and BRCA2 and a propensity to PanNETs in approximately 17% of the studied population [1]. 2. Diagnostics 2.1. Biochemical diagnostics Biochemical diagnostics of NENs involves the following: A. Non-specific markers The most frequently used diagnostic method is determination of the chromogranin A (CgA) concentration in the serum (less frequently in the plasma) [1, 8, 9]. CgA is a relatively stable protein in blood. However, there are two different methods for determining the concentration of CgA: radioimmunoassay (RIA) and enzyme-linked immunosorbent assay (ELISA) in Neuroendocrine neoplasms (NENs) arise from the disseminated system of neuroendocrine cells and can occur in various parts of the body. However, they are most often found in the gastrointestinal tract and lungs. The term NENs includes both well-differentiated neuroendocrine tumours and neuroendocrine carcinomas (NECs), which account for 10-20% of all NENs. The following characteristics of NENs should be considered in the diagnostic and therapeutic process: proliferative activity, presence of somatostatin receptors (SSTRs), tumour growth rate, and extent of the neoplastic disease [1]. in the prevalence of GEP-NENs. Local and regional NENs are diagnosed more frequently than those with distant metastases. The detectability of NENs is increasing, e.g. from 1973 to 2004 the incidence of NENs increased from 2.1 to 5.25 new cases per 100,000

2014

Diagnostic workup Á Markers Á Imaging Á Incidental findings Á Nonfunctioning tumors Á Carcinoid syndrome Á Gastrinoma Á Insulinoma Á NET Á NEC Á NEN Abbreviations 5-HIAA 5-Hydroxy-indolacetic acid ACE Angiotensin-converting enzyme ACTH Adrenocorticotropin AJCC American Joint Committee on Cancer AKT A protein-serine-threonine kinase that is activated by phosphorylation in response to growth factors or insulin CD117 Antigen specific for the proto-oncogene c-kit CD56 Antigen expressed by all lymphocytes CD99 Cluster of differentiation CDX-2 Transcription factor expressed specifically in gut epithelium CEACAM1 Cell adhesion molecule CEUS Contrast-enhanced US CgA Chromogranin A CK19 Cytokeratin 19 CS Carcinoid syndrome CT Computerized tomography DBE Double balloon enteroscopy On behalf of AME. Other members of AME oncologic endocrinology group are listed in the conclusions.

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