scholarly journals Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: an analysis based on prospectively collected parameters

2010 ◽  
Vol 17 (4) ◽  
pp. 909-918 ◽  
Author(s):  
Martin B Niederle ◽  
Monika Hackl ◽  
Klaus Kaserer ◽  
Bruno Niederle
Keyword(s):  
Digestive Tract ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Tnm Staging ◽  
Incidence Rates ◽  
Neuroendocrine Cells ◽  
Malignant Tumours ◽  
Main Site ◽  
Well Differentiated ◽  
Gastroenteropancreatic Neuroendocrine Tumours

As incidence data on gastroenteropancreatic neuroendocrine tumours (GEP-NETs) have so far only been retrospectively obtained and based on inhomogeneous material, we conducted a prospective study in Austria collecting all newly diagnosed GEP-NETs during 1 year. Using the current WHO classification, the tumor, nodes, metastases (TNM) staging and Ki67 grading and the standard diagnostic procedure proposed by the European Neuroendocrine Tumor Society (ENETS), GEP-NETs from 285 patients (male: 148; female: 137) were recorded. The annual incidence rates were 2.51 per 100 000 inhabitants for men, 2.36 per 100 000 for women. The stomach (23%) was the main site, followed by appendix (21%), small intestine (15%) and rectum (14%). Patients with appendiceal tumours were significantly younger than patients with tumours in any other site. About 46.0% were classified as benign, 15.4% as uncertain, 31.9% as well differentiated malignant and 6.7% as poorly differentiated malignant. Patients with benign or uncertain tumours were significantly younger than patients with malignant tumours. Among the malignant tumours of the digestive tract, 1.49% arose from neuroendocrine cells. For malignant gastrointestinal NETs, the incidence was 0.80 per 100 000: 40.9% were ENETS stage I, 23.8% stage II, 11.6% stage III and 23.8% stage IV. The majority (59.7%) were grade 1, 31.2% grade 2 and 9.1% grade 3. NETs of the digestive tract are more common than previously reported; the majority show benign behaviour, are located in the stomach and are well differentiated. G3 tumours are very rare.

scholarly journals Diagnosis and Prognosis of Neuroendocrine Tumours of the Lung by Means of High Resolution Image Analysis

1999 ◽  
Vol 18 (2) ◽  
pp. 109-119 ◽  
Author(s):  
Uta Jütting ◽  
Peter Gais ◽  
Karsten Rodenacker ◽  
Joachim Böhm ◽  
Susanne Koch ◽  
...  
Keyword(s):  
Image Analysis ◽  
High Resolution ◽  
Neuroendocrine Tumours ◽  
Malignant Potential ◽  
Low Grade ◽  
Malignant Tumours ◽  
Cox Regression Analysis ◽  
Resolution Image ◽  
High Resolution Image ◽  
Well Differentiated

Neuroendocrine tumours (NET) of the lung are divided in subtypes with different malignant potential. The first is the benign or low‐grade malignant tumours, well‐differentiated, called typical carcinoids (TC) and the second is the high‐grade malignant tumours, poorly differentiated of small (SCLC) or large cell type (LCLC). Between these tumour types lies the well‐differentiated carcinoma with a lower grade of malignancy (WDNEC). In clinical routine it is very important with regard to prognosis to distinguish patients with low malignant potential from those with higher ones. In this study 32 cases of SCLC, 13 of WDNEC and 14 of TC with a follow‐up time up to 7 years were collected. Sections 4 μm thick from paraffin embedded tissue were Feulgen stained. By means of high resolution image analysis 100 nuclei per case were randomly gathered to extract morphometric, densitometric and textural quantitative features. To investigate the ploidy status of the tumour the corrected DNA distribution was calculated. Stepwise linear discriminant analysis to differentiate the classes and Cox regression analysis for the survival time analysis were applied. Using chromatin textural and morphometric features in two two‐class discriminations, 11 of the 14 TC cases and 8 of the 13 WDNEC cases were correctly classified and 11/13 WDNEC cases and 28/32 SCLC cases, respectively. The WDNEC cases are more similar in chromatin structure to TC than to SCLC. For the survival analysis, only chromatin features were selected to differentiate patients with better and worse prognosis independent of staging and tumour type.

Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms

2011 ◽  
Vol 18 (S1) ◽  
pp. S1-S16 ◽  
Author(s):  
Günter Klöppel
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Tnm Stage ◽  
Neuroendocrine Neoplasms ◽  
Gastroenteropancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Neuroendocrine Carcinomas

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are composed of cells with a neuroendocrine phenotype. The old and the new WHO classifications distinguish between well-differentiated and poorly differentiated neoplasms. All well-differentiated neoplasms, regardless of whether they behave benignly or develop metastases, will be called neuroendocrine tumours (NETs), and graded G1 (Ki67 <2%) or G2 (Ki67 2–20%). All poorly differentiated neoplasms will be termed neuroendocrine carcinomas (NECs) and graded G3 (Ki67 >20%). To stratify the GEP-NETs and GEP-NECs regarding their prognosis, they are now further classified according to TNM-stage systems that were recently proposed by the European Neuroendocrine Tumour Society (ENETS) and the AJCC/UICC. In the light of these criteria the pathology and biology of the various NETs and NECs of the gastrointestinal tract (including the oesophagus) and the pancreas are reviewed.

scholarly journals The multidisciplinary team for gastroenteropancreatic neuroendocrine tumours: the radiologist’s challenge

2019 ◽  
Vol 53 (4) ◽  
pp. 373-387 ◽  
Author(s):  
Vincenza Granata ◽  
Roberta Fusco ◽  
Sergio Venanzio Setola ◽  
Elisabetta de Lutio di Castelguidone ◽  
Luigi Camera ◽  
...  
Keyword(s):  
Multidisciplinary Team ◽  
Neuroendocrine Tumours ◽  
Clinical Symptoms ◽  
Neuroendocrine Tumour ◽  
Imaging Techniques ◽  
Critical Role ◽  
Nuclear Imaging ◽  
Distant Metastases ◽  
Gastroenteropancreatic Neuroendocrine Tumours ◽  
Effective Diagnosis

Abstract Background Gastroenteropancreatic neuroendocrine tumours (GEP-NETs) are a heterogeneous group of tumours. An effective diagnosis requires a multimodal approach that combines evaluation of clinical symptoms, hormonelevels, radiological and nuclear imaging, and histological confirmation. Imaging plays a critical role in NETs diagnosis, prognosis and management, so the radiologists are important members of the multidisciplinary team. During diagnostic work-up two critical issues are present: firstly the need to identify tumor presence and secondly to define the primary site and assess regional and distant metastases. Conclusions The most appropriate imaging technique depends on the type of neuroendocrine tumour and the availability of specialized imaging techniques and expertise. There is no general consensus on the most efficient imaging pathway, reflecting the challenge in reliably detection of these tumours.

Marked improvement in hyperammonaemic encephalopathy from multimodal treatment of metastatic neuroendocrine tumour

2021 ◽  
Vol 14 (6) ◽  
pp. e241191
Author(s):  
Alexander Davis ◽  
Stephen Clarke ◽  
Sally Ayesa ◽  
David L Chan
Keyword(s):  
Poor Prognosis ◽  
Neuroendocrine Tumours ◽  
Treatment Approach ◽  
Neuroendocrine Tumour ◽  
Multimodality Treatment ◽  
Peptide Receptor ◽  
The Poor ◽  
Heterogenous Group ◽  
Gastroenteropancreatic Neuroendocrine Tumours ◽  
New Diagnosis

Gastroenteropancreatic neuroendocrine tumours (GEPNETs) are a heterogenous group of tumours which are rising in incidence. Morbidity and mortality related to these tumours is dependent on the location of metastatic spread. Hyperammonaemia and subsequent encephalopathy has previously been described in GEPNET and is typically associated with a poor prognosis. We describe a case of a 55-year-old woman with hyperammonaemic encephalopathy and a new diagnosis of GEPNET. Given the poor prognosis and the outcomes in this patient group we feel this case highlights the benefit of a multimodality treatment approach including peptide receptor radionucleotide therapy and transarterial chemoembolisation.

scholarly journals Incidence of gastroenteropancreatic neuroendocrine tumours: a systematic review of the literature

2013 ◽  
Vol 21 (3) ◽  
pp. R153-R163 ◽  
Author(s):  
M Fraenkel ◽  
M Kim ◽  
A Faggiano ◽  
W W de Herder ◽  
G D Valk ◽  
...  
Keyword(s):  
Racial Differences ◽  
Western Europe ◽  
Neuroendocrine Tumours ◽  
Cancer Registries ◽  
Incidence Rates ◽  
Anatomical Site ◽  
Cascade Process ◽  
Medline Search ◽  
The Usa ◽  
Gastroenteropancreatic Neuroendocrine Tumours

Based on the current medical literature, the worldwide incidence of neuroendocrine tumours (NETs) seems to have increased; however, a systematic literature overview is lacking. This study aimed to collect all available data on the incidence of gastroenteropancreatic (GEP)-NETs and characteristics of population to establish their epidemiology. A sensitive MEDLINE search was carried out. The papers were selected via a cascade process that restricted the initial pool of 7991 articles to 33, using predefined inclusion and exclusion criteria. Original articles evaluating the incidence of sporadic GEP-NETs in regional, institutional and national registries were considered. The majority of data originated from the US National Cancer Institute Surveillance, Epidemiology and End Results database and from national cancer registries in Western Europe. Generally, because of the retrospective nature of existing databases the outcomes of studies might be biased, which hinders the drawing of firm conclusions. The age-adjusted incidence of GEP-NETs has increased steadily over the past four decades (1973–2007), increasing 3.65-fold in the USA and 3.8- to 4.8-fold in the UK. Incidence has changed variably from one anatomical site to another. The greatest increase in incidence occurred for gastric and rectal NETs, while the smallest increase occurred for small intestine NETs. There were gender and racial differences, which differed site by site and, in some cases, changed over time. The incidence rates (IRs) of GEP-NETs have increased significantly in the last 40 years. Data are only available from North America, Western Europe and Japan. A site-by-site analysis revealed that the IRs of some NETs increased more than those of others.

scholarly journals Requiem for the Term ‘Carcinoid Tumour’ in the Gastrointestinal Tract?

2008 ◽  
Vol 22 (4) ◽  
pp. 357-358 ◽  
Author(s):  
Runjan Chetty
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Carcinoma ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Carcinoid Tumour ◽  
World Health ◽  
Low Grade ◽  
Biological Behaviour ◽  
Unique Type ◽  
Well Differentiated

Use of the term ‘carcinoid tumour’ to describe a unique type of tumour in the gastroenteropancreatic system is endemic in the medical literature and in daily clinical and pathological parlance. However, it is a somewhat misleading moniker because a spectrum of histopathological changes and hence, biological outcomes may occur in these tumours. The World Health Organization classification scheme recommends the use of the terms neuroendocrine tumours or carcinomas, which may be stratified as well-differentiated neuroendocrine tumours with benign or uncertain behaviour, well-differentiated tumours with low-grade neuroendocrine carcinoma behaviour and high-grade neuroendocrine carcinomas. These categories may be applied within different sites in the gastrointestinal tract and pancreas, and convey a sense of biological behaviour. In addition, a recently suggested tumour-node-metastasis scheme has been proposed and awaits clinical validation and acceptance. Thus, the term ‘carcinoid’ has served its purpose well, but its use should be phased out in favour of ‘neuroendocrine tumour’ or ‘neuroendocrine carcinoma’.

scholarly journals Exploring the current status of neuroendocrine tumours: a population-based analysis of epidemiology, management and use of resources

BMC Cancer ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Josep Darbà ◽  
Alicia Marsà
Keyword(s):  
Disease Management ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Fold Increase ◽  
Current Status ◽  
Metastatic Tumour ◽  
Neuroendocrine Neoplasms ◽  
Use Of Resources ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Well Differentiated

Abstract Background Neuroendocrine tumours (NETs) are rare malignancies characterised by its capacity to synthesise and secrete monoamines, due to its neuroendocrine origin. Its varied locations and symptoms have traditionally been responsible for extended delays in their diagnosis. The interest of this study was to characterise the patient population diagnosed with NETs in Spain and to revise how the disease is managed, together with the hospitalisation costs of these patients. Methods The database included records of all patients diagnosed with a NET between 2010 and 2015. Admission records were used to evaluate hospitalisation, disease management data and costs, and single-patient files were used to characterise the population. Results Nine Thousand One Hundred Twenty patients were diagnosed with a neuroendocrine tumour between 2010 and 2015, with a 2 fold increase in the diagnosis rate over the study period. 42.25% of the patients were females, while 57.75% were males, and mean diagnosis age was 62.58 years (SD = 14.65). Considering all the registered neuroendocrine neoplasms, 46.86% of the patients had malignant well-differentiated NETs, 32.02% had a malignant poorly differentiated neuroendocrine carcinoma and 42.93% of patients developed metastatic NETs. In addition, 18.59% of patients were diagnosed with benign well-differentiated NETs. The most common tumour sites were the bronchus, lung and other sites, including pancreatic tumours; metastasis was found in the liver and distant lymph nodes. Pancreatic resection was the most common surgical procedure utilised in these patients, summing 19% of total expenses, the injection of an unspecified therapeutic substance (including targeted therapies) was registered in 11.40% of admissions, while chemotherapy was registered in only 6.85% of admissions. The annual healthcare cost of NETs was €15,373,961, corresponding to €9092 per patient. Conclusions The implementation of standard diagnosis procedures should be prioritised, with a focus on the pancreas and lung, and taking into account that 42.93% of the patients develop a metastatic tumour. The presence of comorbidities and multimorbidities should be considered in order to develop more efficient disease management protocols.

Neuroendocrine tumours.

2004 ◽  
Vol 11 (1) ◽  
pp. 1-18 ◽  
Author(s):  
M T Barakat ◽  
K Meeran ◽  
S R Bloom
Keyword(s):  
Neuroendocrine Tumours ◽  
Secretory Granules ◽  
Symptom Control ◽  
Medullary Carcinoma ◽  
Somatostatin Analogues ◽  
Pharmacological Therapy ◽  
Neuroendocrine Cells ◽  
Hormone Production ◽  
Neuropeptide Hormone ◽  
Gastroenteropancreatic Neuroendocrine Tumours

Neuroendocrine tumours are a heterogeneous group including, for example, carcinoid, gastroenteropancreatic neuroendocrine tumours, pituitary tumours, medullary carcinoma of the thyroid and phaeochromocytomas. They have attracted much attention in recent years, both because they are relatively easy to palliate and because they have indicated the chronic effect of the particular hormone elevated. As neuroendocrine phenotypes became better understood, the definition of neuroendocrine cells changed and is now accepted as referring to cells with neurotransmitter, neuromodulator or neuropeptide hormone production, dense-core secretory granules, and the absence of axons and synapses. Neuroendocrine markers, particularly chromogranin A, are invaluable diagnostically. Study of several neuroendocrine tumours has revealed a genetic etiology, and techniques such as genetic screening have allowed risk stratification and prevention of morbidity in patients carrying the particular mutation. Pharmacological therapy for these often slow-growing tumours, e.g. with somatostatin analogues, has dramatically improved symptom control, and radiolabelled somatostatin analogues offer targeted therapy for metastatic or inoperable disease. In this review, the diagnosis and management of patients with carcinoid, gut neuroendocrine tumours, multiple endocrine neoplasia types 1 and 2, and isolated phaeochromocytoma are evaluated.

scholarly journals Not only stem cells, but also mature cells, particularly neuroendocrine cells, may develop into tumours: time for a paradigm shift

2018 ◽  
Vol 11 ◽  
pp. 175628481877505 ◽  
Author(s):  
Helge L. Waldum ◽  
Kjell Öberg ◽  
Øystein F. Sørdal ◽  
Arne K. Sandvik ◽  
Bjørn I. Gustafsson ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Cells ◽  
Cell Of Origin ◽  
Malignant Tumours ◽  
Cellular Origin ◽  
Degree Of Malignancy ◽  
Benign Tumours

Stem cells are considered the origin of neoplasms in general, and malignant tumours in particular, and the stage at which the stem cells stop their differentiation determines the degree of malignancy. However, there is increasing evidence supporting an alternative paradigm. Tumours may develop by dedifferentiation from mature cells able to proliferate. Studies of gastric carcinogenesis demonstrate that mature neuroendocrine (NE) cells upon long-term overstimulation may develop through stages of hyperplasia, dysplasia, and rather benign tumours, into highly malignant carcinomas. Dedifferentiation of cells may change the histological appearance and impede the identification of the cellular origin, as seen with gastric carcinomas, which in many cases are dedifferentiated neuroendocrine tumours. Finding the cell of origin is important to identify risk factors for cancer, prevent tumour development, and tailor treatment. In the present review, we focus not only on gastric tumours, but also evaluate the role of neuroendocrine cells in tumourigenesis in two other foregut-derived organs, the lungs and the pancreas, as well as in the midgut-derived small intestine.

scholarly journals Well-Differentiated Grade 2, Type 3 Gastrointestinal Neuroendocrine Tumour with Bilateral Metastatic Ovarian Involvement: Report of an Unusual Case

2016 ◽  
Vol 9 (1) ◽  
pp. 255-261 ◽  
Author(s):  
Ray Manneh ◽  
Daniel Castellano ◽  
Oscar Caso ◽  
Carmelo Loinaz ◽  
Jesús Jiménez ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Adjuvant Treatment ◽  
Unusual Case ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Somatostatin Analogues ◽  
Low Grade ◽  
Well Differentiated ◽  
Type 3 ◽  
Ovarian Involvement

Treatment of metastatic gastric neuroendocrine tumours (NETs) is challenging. In oligometastatic cases, surgical resection is recommended whenever possible. Somatostatin analogues have been used to decrease gastrin levels, and available evidence suggests that these drugs can also reduce recurrences. Here we present a highly unusual case involving a patient with a well-differentiated grade 2, type 3 gastric NET with exclusive metastatic bilateral ovarian involvement. To our knowledge, this is the first such case reported in the literature, as the cause of ovarian involvement is usually due to local invasion rather than metastasis. We believe this case is of interest not only due to the unusual presentation, but also because it makes us consider adjuvant treatment with somatostatin analogues in patients with low-grade tumours and a positive postoperative octreoscan.

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