Hepatic arterial embolisation for patients diagnosed with neuroendocrine neoplasms: experience from a European Neuroendocrine Tumour Society centre of excellence

2019 ◽  
Author(s):  
Christina Nuttall ◽  
Angela Lamarca ◽  
Mairead McNamara ◽  
Jorge Barriuso ◽  
Jeremy Lawrance ◽  
...  
Keyword(s):  
Neuroendocrine Tumour ◽  
Neuroendocrine Neoplasms ◽  
Arterial Embolisation ◽  
Centre Of Excellence

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 Paranasal Sinus Neuroendocrine Carcinoma: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Nagesh T. Sirsath ◽  
K. Govind Babu ◽  
Umesh Das ◽  
C. S. Premlatha
Keyword(s):  
Paranasal Sinus ◽  
Neuroendocrine Carcinoma ◽  
Neuroendocrine Tumour ◽  
Neuroendocrine Differentiation ◽  
Complete Response ◽  
The Body ◽  
Intracranial Extension ◽  
Neuroendocrine Neoplasms ◽  
Rare Site ◽  
Poorly Differentiated

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 sinuses have been reported to be recurrent and locally destructive. Very few cases of paranasal sinus neuroendocrine carcinoma have been reported till date. Difficulty in pathologic diagnosis and rarity of this malignancy have hindered the progress in understanding the clinical course and improving outcomes. We herein report a case of poorly differentiated neuroendocrine tumour of ethmoid and sphenoid sinus with invasion of orbit and intracranial extension. The patient had complete response at the end of chemoradiation and he was disease-free for 9 months duration after which he developed bone metastasis without regional recurrence.

Neuroendocrine neoplasms of the pancreas at dynamic enhanced CT: comparison between grade 3 neuroendocrine carcinoma and grade 1/2 neuroendocrine tumour

2014 ◽  
Vol 25 (5) ◽  
pp. 1375-1383 ◽  
Author(s):  
Dong Wook Kim ◽  
Hyoung Jung Kim ◽  
Kyung Won Kim ◽  
Jae Ho Byun ◽  
Ki Byung Song ◽  
...  
Keyword(s):  
Neuroendocrine Carcinoma ◽  
Neuroendocrine Tumour ◽  
Neuroendocrine Neoplasms ◽  
Enhanced Ct ◽  
Grade 3

scholarly journals P124 Gastroenteropancreatic Neuroendocrine Neoplasms in patients with Inflammatory Bowel Disease: An ECCO CONFER Multicentre Case Series

2021 ◽  
Vol 15 (Supplement_1) ◽  
pp. S215-S216
Author(s):  
S Festa ◽  
G Zerboni ◽  
L Derikx ◽  
D G Ribaldone ◽  
G Dragoni ◽  
...  
Keyword(s):  
Neuroendocrine Tumour ◽  
Case Reports ◽  
Case Series ◽  
The Body ◽  
Neuroendocrine Neoplasms ◽  
Case Report Form ◽  
Related Factors ◽  
Common Location ◽  
Inflammatory Bowel

Abstract Background Neuroendocrine Neoplasms (NENs) are a heterogeneous group of tumours deriving from the diffuse endocrine system. NENs may occur almost everywhere in the body but are most common in the gastrointestinal tract, the pancreas, and the lungs, with gastroenteropancreatic (GEP) tumours representing 70% of all NENs. GEP-NENs have rarely been reported in association with inflammatory bowel diseases (IBDs) but no definitive relationship between these tumours and IBD has been established Methods This was an ECCO COllaborative Network For Exceptionally Rare case reports project (ECCO-CONFER). We included cases of GEP-NENs diagnosed in patients with IBD that met the diagnostic criteria for NEN according to the European Neuroendocrine Tumour Society. Data were retrospectively collected in a standardized case report form and analysed for event association with patient’s and IBD-related factors Results GEP-NEN was diagnosed in 100 patients with IBD [61% female, 55% Crohn’s disease, median age 48 years (IQR 37–59)]. Overall the most common location was the appendix (39/100) followed by the colon (22/100). Complete IBD-related data was available for 50 individuals with a median follow-up of 30.5 months (IQR 11.2–70) following NEN diagnosis. At the last follow-up data, 47/50 patients were alive. Three deaths occurred, of which 2 were related to NEN. Median duration of IBD at NEN diagnosis was 84 months (IQR 10–151), and in 18% of cases NEN and IBD were diagnosed concomitantly. 20/50 of NENs were at stage I (T1N0M0) and 28/50 graded G1 (ki 67 ≤2 %) at diagnosis. Incidental diagnosis of NEN either during follow-up or during surgery as well as receiving diagnosis of NEN concomitantly with IBD was significantly associated with an earlier NEN stage (p&lt; 0.01 and p&lt;0.02, respectively). Exposure to immunomodulatory and/or biologic therapy was not associated with advanced NEN stage or grade. Interestingly, primary GEP-NEN sites significantly correlated to the segment affected by IBD (62% vs 38% p = 0.02) Conclusion In the largest case series to date, prognosis of patients with concomitant GEP-NEN and IBD seems favorable. Incidental NEN diagnosis correlates with an earlier NEN stage and IBD-related therapies are independent of NEN stage and grade. The association of GEP-NEN location and the segment affected by IBD may suggest a possible role of inflammation in NEN tumorigenesis

Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) of pancreas: a rare entity—worth to note

2020 ◽  
Vol 13 (4) ◽  
pp. e234855 ◽  
Author(s):  
Bharti Varshney ◽  
Jyotsna Naresh Bharti ◽  
Vaibhav Kumar Varshney ◽  
Taruna Yadav
Keyword(s):  
Histopathological Examination ◽  
Neuroendocrine Tumour ◽  
Pancreatic Head ◽  
Ductal Adenocarcinoma ◽  
Neuroendocrine Neoplasms ◽  
Contrast Enhanced Ct ◽  
Contrast Enhanced ◽  
Enhanced Ct ◽  
Rare Malignancy

Mixed adenocarcinoma with neuroendocrine tumour of pancreas has been reported infrequently and consists of both epithelial and neuroendocrine component. We encountered an 81-year-old male patient who presented with clinical features of painful progressive jaundice for 1 month. Contrast-enhanced CT abdomen reported a mass in the pancreatic head with dilated common bile duct and pancreatic duct. He underwent pancreatoduodenectomy and histopathological examination revealed two different tumours: ductal adenocarcinoma admixed with neuroendocrine tumour of pancreas. He received adjuvant chemotherapy, and at the end of 1-year follow-up, he has no recurrence. Here, we reported this rare malignancy of pancreas for which pancreatoduodenectomy was done and diagnosed on histopathology with immunohistochemistry.

scholarly journals Effect of Octreotide Long-Acting Release on Tregs and MDSC Cells in Neuroendocrine Tumour Patients: A Pivotal Prospective Study

Cancers ◽  
2020 ◽  
Vol 12 (9) ◽  
pp. 2422
Author(s):  
Claudia von Arx ◽  
Giuseppina Rea ◽  
Maria Napolitano ◽  
Alessandro Ottaiano ◽  
Fabiana Tatangelo ◽  
...  
Keyword(s):  
Immune System ◽  
Prospective Study ◽  
Neuroendocrine Tumour ◽  
Hormone Secretion ◽  
Somatostatin Receptors ◽  
Growth Hormone Secretion ◽  
Suppressor Cells ◽  
Neuroendocrine Neoplasms ◽  
Long Acting ◽  
The Impact

Octreotide long-acting repeatable (LAR) is largely used to treat functional and/or metastatic neuroendocrine neoplasms (NENs). Its effect in controlling carcinoid syndrome and partially reduce tumour burden is attributable to the ability of octreotide to bind somatostatin receptors (SSTRs) on the tumour and metastasis, regulating growth hormone secretion and cell growth. Notably, SSTRs are also expressed, at different levels, on Tregs. Tregs, together with myeloid-derived suppressor cells (MDSCs), are key components in the anti-tumour immunoregulation. This is the first prospective study aimed to explore the impact of Octreotide (OCT) LAR on the immune system, with a particular focus on Tregs and MDSC cells. Here, we show that circulating Tregs are elevated in NENs patients compared to healthy donors and that treatment with OCT LAR significantly decrease the level of total Tregs and of the three functional Tregs populations: nTregs, eTregs and non-Tregs. Furthermore, OCT LAR treatment induces a functional impairment of the remaining circulating Tregs, significantly decreasing the expression of PD1, CTLA4 and ENTPD1. A trend in circulating MDSC cells is reported in patients treated with OCT LAR. The results reported here suggest that the effect of OCT LAR on Tregs could tip the balance of the patients’ immune-system towards a durable anti-tumour immunosurveillance with consequent long-term control of the NENs disease.

Neuroendocrine tumour of the ampulla of Vater: a rare neoplasm at an atypical site

2020 ◽  
pp. 1-10
Author(s):  
Abrar Zahid ◽  
Danish Ali ◽  
Muhammad Zubair ◽  
Irfan Ahmed ◽  
Tauseef Fatima ◽  
...  
Keyword(s):  
Weight Loss ◽  
Immunohistochemical Staining ◽  
Poor Prognosis ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumour ◽  
Carcinoid Tumour ◽  
Treatment Options ◽  
Ampulla Of Vater ◽  
Neuroendocrine Neoplasms ◽  
Late Event

Abstract The periampullary neuroendocrine tumour is an infrequently occurring tumour. Its prevalence among gastrointestinal neuroendocrine neoplasms is less than 0.3%, and less than 2% out of periampullary tumours. These neoplasms have relatively poor prognosis. Jaundice and pain in the abdomen are the early and most commonly occurring symptoms with weight loss being a late event. The carcinoid syndrome presents infrequently in periampullary neuroendocrine tumour and happens only if hepatic metastasis occurs. In this scenario, histopathology plays a paramount role in the diagnosis. Specific immunohistochemical staining is used for diagnosis while the treatment options are local excision, endoscopic excision and pancreaticoduodenectomy. Here is a case report of a 42-year-old patient who presented with complaint of obstructive jaundice for one month. Periampullary carcinoid tumour was diagnosed on biopsy, and she underwent Pancreaticoduodenectomy as treatment. Continuou...  

scholarly journals Carcinoid syndrome: Patient outcomes from a European Neuroendocrine Tumour Society (ENETs) centre of excellence

2017 ◽  
Vol 28 ◽  
pp. v148 ◽  
Author(s):  
P.D. d'Arienzo ◽  
E. Amir ◽  
A.R. Lewis ◽  
L. Magdalani ◽  
W. Mansoor ◽  
...  
Keyword(s):  
Patient Outcomes ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumour ◽  
Syndrome Patient ◽  
Centre Of Excellence

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.

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