Management of Neuroendocrine Tumors of the Pancreas

2018 ◽  
Author(s):  
Robert CG Martin II
Keyword(s):  
Neuroendocrine Tumors ◽  
Time Management ◽  
Treatment Options ◽  
Life Time ◽  
Tumor Grade ◽  
Islet Cell Carcinoma ◽  
Key Words Carcinoid Tumor ◽  
Well Differentiated

Neuroendocrine tumors (NETs) comprise a heterogeneous group of malignancies, with differences in prognosis and effective therapies. Traditionally, NETs have been characterized by tumor grade, site of primary tumor, functional status, and presence of underlying familial syndrome. However, increased feasibility and use of next-generation sequencing and other methodologies have revealed new genomic and epigenetic aberrations. In the past decade, treatment options available for metastatic well-differentiated gastroenteropancreatic NETs have expanded, with the approval of antiangiogenic and mTOR-directed targeted therapies, and our armamentarium of active therapies is likely to further increase. Optimal surgical therapy both for nonfunctional and functional PNETs has become more personalized and strategic to optimize long-term quality of life time. Management of localized metastatic PNET has become a true multidisciplinary management opportunity that all treating physicians should use. Peptide receptor radioligand therapy is an emerging treatment that uses functional imaging to personalize dosimetry to the tumor and avoid nephrotoxicity. Nevertheless, there is a critical need for further biomarkers, particularly multianalyte biomarkers, to aid in prognostication and predict efficacy of therapies. This review contains 1 figure, 1 table, and 72 references. Key Words: carcinoid tumor, islet cell carcinoma, neuroendocrine tumors, pancreatic, surgical management, medical management, metastatic, localized.

Management of Neuroendocrine Tumors of the Pancreas

2018 ◽  
Author(s):  
Robert CG Martin II
Keyword(s):  
Neuroendocrine Tumors ◽  
Time Management ◽  
Treatment Options ◽  
Life Time ◽  
Tumor Grade ◽  
Islet Cell Carcinoma ◽  
Key Words Carcinoid Tumor ◽  
Well Differentiated

Neuroendocrine tumors (NETs) comprise a heterogeneous group of malignancies, with differences in prognosis and effective therapies. Traditionally, NETs have been characterized by tumor grade, site of primary tumor, functional status, and presence of underlying familial syndrome. However, increased feasibility and use of next-generation sequencing and other methodologies have revealed new genomic and epigenetic aberrations. In the past decade, treatment options available for metastatic well-differentiated gastroenteropancreatic NETs have expanded, with the approval of antiangiogenic and mTOR-directed targeted therapies, and our armamentarium of active therapies is likely to further increase. Optimal surgical therapy both for nonfunctional and functional PNETs has become more personalized and strategic to optimize long-term quality of life time. Management of localized metastatic PNET has become a true multidisciplinary management opportunity that all treating physicians should use. Peptide receptor radioligand therapy is an emerging treatment that uses functional imaging to personalize dosimetry to the tumor and avoid nephrotoxicity. Nevertheless, there is a critical need for further biomarkers, particularly multianalyte biomarkers, to aid in prognostication and predict efficacy of therapies. This review contains 1 figure, 1 table, and 72 references. Key Words: carcinoid tumor, islet cell carcinoma, neuroendocrine tumors, pancreatic, surgical management, medical management, metastatic, localized.

Thrombopoietin Receptor Agonists in Children with Immune Thrombocytopenia: A New Therapeutic Era

2020 ◽  
Vol 20 ◽  
Author(s):  
Giuseppe Lassandro ◽  
Valentina Palladino ◽  
Giovanni Carlo Del Vecchioa ◽  
Viviana Valeria Palmieri ◽  
Paola Carmela Corallo ◽  
...  
Keyword(s):  
Immune Thrombocytopenia ◽  
Treatment Options ◽  
Thrombopoietin Receptor Agonists ◽  
Receptor Agonists ◽  
Thrombopoietin Receptor ◽  
Related Quality ◽  
Health Related

Background and Objective: Immune thrombocytopenia (ITP) is a common bleeding disorder in childhood. The management of ITP in children is controversial, requiring personalized assessment of patients and therapeutic choices. Thrombopoietin receptor agonists (TPO-RAs), eltrombopag and romiplostim, have been shown to be safety and effective for the treatment of pediatric ITP. The aim of our research is defining the role of thrombopoietin receptor agonists in the management of pediatric ITP. Method: This review focuses on the use of TPO-RAs in pediatric ITP, in randomized trials and in clinical routine, highlighting their key role in management of the disease. Results: Eltrombopag and romiplostim appear effective treatment options for children with ITP. Several clinical studies have assessed that the use of TPO-RAs increases platelet count, decreases bleeding symptoms and improves health-related quality of life. Moreover, TPO-RAs are well tolerated with minor side effects. Conclusion: Although TPO-RAs long term efficacy and safety still require further investigations, their use is gradually expanding in clinical practice of children with ITP.

Prognostic transcriptome classes of duodenopancreatic neuroendocrine tumors

2021 ◽  
Author(s):  
Marc Diedisheim ◽  
Solène Dermine ◽  
Anne Jouinot ◽  
Amandine Septier ◽  
Sébastien Gaujoux ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Pancreatic Beta Cell ◽  
Tumor Biology ◽  
Disease Free Survival ◽  
Tumor Grade ◽  
Pancreatic Alpha Cell ◽  
Liver Gene ◽  
Well Differentiated ◽  
Grade 3 ◽  
Definition Of

Duodenopancreatic neuroendocrine tumors (DPNETs) aggressiveness is heterogeneous. Tumor grade and extension are commonly used for prognostic determination. Yet, grade classes are empirically defined, with regular up-dates changing the definition of classes. Genomic screening may provide more objective classes, and reflect tumor biology. The aim of this study was to provide a transcriptome classification of DPNETs. We included 66 DPNETs, covering the entire clinical spectrum of the disease in terms of secretion, grade, and stage. Three distinct molecular groups were identified, associated with distinct outcome (log-rank p<0.01): (i) better-outcome DPNETs with pancreatic beta-cell signature. This group was mainly composed of well-differentiated, grade 1 insulinomas; (ii) poor-outcome DPNETs with pancreatic alpha-cell and hepatic signature. This group included all neuroendocrine carcinomas and grade 3 DPNETs, but also some grade 1 and grade 2 DPNETs; and (iii) intermediate-outcome DPNETs with pancreatic exocrine and progenitor signature. This group included grade 1 and grade 2 DPNETs, with some insulinomas. Fibrinogen gene FGA expression was one of the top most expressed liver gene. FGA expression was associated with disease-free survival (HR=1.13, p=0.005), and could be validated on two independent cohorts. This original pathophysiologic insight provides new prognostic classification perspectives.

scholarly journals Bilateral adrenalectomy in Cushing's disease: Altered long-term quality of life compared to other treatment options

2019 ◽  
Vol 80 (1) ◽  
pp. 32-37 ◽  
Author(s):  
Pauline Sarkis ◽  
Muriel Rabilloud ◽  
Jean-Christophe Lifante ◽  
Anna Siamand ◽  
Emmanuel Jouanneau ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cushing’S Disease ◽  
Treatment Options ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease

scholarly journals Changes of Insulation Properties of Photovoltaic Cables Caused by Ageing Treatment

2020 ◽  
Vol 6 (1) ◽  
pp. 6-9 ◽  
Author(s):  
Juraj Packa ◽  
Vladimir Kujan ◽  
Daniel Štrkula ◽  
Vladimír Šály ◽  
Milan Perný
Keyword(s):  
Power Plants ◽  
Real Life ◽  
Life Time ◽  
Operating Conditions ◽  
Solar Panels ◽  
Font Size ◽  
Long Term Stability ◽  
Cable Systems

<span style="font-family: 'Times New Roman',serif; font-size: 10pt; mso-fareast-font-family: 'Times New Roman'; mso-ansi-language: EN-US; mso-fareast-language: EN-US; mso-bidi-language: AR-SA;" lang="EN-US">An important part of the photovoltaic power plants are cable systems. The dielectric properties of cables, reliability and durability depend on quality of production processes, operating conditions and degradation factors, as well. Expected lifetime of cable systems is more than 20-30 years in general. Their failure free operation and long-term stability of properties has a direct impact on the economic return of the investments. According to our experiences the tests in compliance with valid standards are not adequate to verify real life time during operation. Photovoltaic cables intended for use in outdoor applications for the connection between the solar panels and possible connection between panels and inverter were chosen for our experiments. <span style="-ms-layout-grid-mode: line;">The changes </span>of insulation resistance and breakdown voltage caused by some degradation factors, mainly water, are presented. This research was inspired by real failure in operation.</span>

Development of a novel scoring system based on endoscopic appearance for management of rectal neuroendocrine tumors

Endoscopy ◽  
2020 ◽  
Author(s):  
Luohai Chen ◽  
Yu Guo ◽  
Yixuan Zhang ◽  
Man Liu ◽  
Yu Zhang ◽  
...  
Keyword(s):  
Overall Survival ◽  
Neuroendocrine Tumors ◽  
Scoring System ◽  
Advanced Disease ◽  
Tumor Grade ◽  
Training Set ◽  
Endoscopic Appearance ◽  
Well Differentiated ◽  
The Relationship ◽  
Disease Free

Abstract Background The clinical significance of the endoscopic appearance of rectal neuroendocrine tumors (NETs) is poorly understood. We aimed to develop a novel scoring system based on endoscopic appearances to predict endoscopically advanced disease in patients with rectal NETs when initially diagnosed. Methods Patients diagnosed with well-differentiated rectal NETs between January 2005 and December 2019 were retrospectively included. Logistic regression analyses were applied to study the relationship between endoscopic appearance and advanced disease. The whole dataset was randomly divided into training and validation sets, which were used to develop and validate a novel scoring system, respectively. Results 309 patients were included. The endoscopic appearance of rectal NETs was significantly associated with advanced disease (P < 0.001). A novel scoring system was developed based on endoscopic appearance, including tumor size, tumor shape, and mucosal surface, using the training set. The area under curve (AUC) of the scoring system to predict advanced disease was 0.953 (95 % confidence interval [CI] 0.915 – 0.991; P < 0.001) and 0.960 (95 %CI 0.905 – 1.000; P < 0.001) in the training and validation sets, respectively. Furthermore, the scoring system was significantly associated with tumor grade. Patients with high scores had significantly worse disease-free and overall survival than patients with low scores (P < 0.001). Conclusion This novel scoring system based on the endoscopic appearance of the primary tumor can help to accurately identify patients with endoscopically advanced disease who are not suitable for endoscopic resection. In addition, it is of great value in monitoring tumor recurrence and overall survival in patients with rectal NETs.

A multi-transcript blood signature for detection and assessment of gut neuroendocrine tumors.

2014 ◽  
Vol 32 (3_suppl) ◽  
pp. 216-216 ◽  
Author(s):  
Mark S. Kidd ◽  
Daniele Alaimo ◽  
Stephen Callahan ◽  
Nancy Smith Texeira ◽  
Ignat Drozdov ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Stable Disease ◽  
Treatment Efficacy ◽  
Progressive Disease ◽  
Performance Metrics ◽  
Treatment Options ◽  
High Sensitivity ◽  
Similar Efficacy ◽  
Metastatic Lesions

216 Background: Gut neuroendocrine tumors (NETs or “carcinoids”) are difficult to manage since they present with advanced disease and limited treatment options are available. Imaging is relatively insensitive in identifying treatment efficacy and the current biomarker, a neurosecretory peptide, Chromogranin A (CgA), has limited value. There is a lack of specific blood biomarker tests both to detect tumors and measure treatment responsiveness. We report the utility of a 51 marker peripheral blood signature (NETest) in comparison to CgA. Methods: The signature was validated using a 2-step qPCR method for detecting NETs in two sets (n=115; n=120) and for measuring treatment responses in a third set (n=133, including complete remission: n=4, clinically stable disease: n=82 and non-responders/clinically progressive disease: n=47). Comparison with CgA (DAKO-ELISA) was undertaken. The confounding effects of PPIs, age, sex and race were also determined for the PCR test. Results: The PCR NETest detected NETs with high sensitivity (85-98%) and specificity (93-97%). It identified pancreatic and gastrointestinal NETs with similar efficacy (>85%) as well as metastatic and non-metastatic lesions. NETest score was significantly reduced (p<0.004) following surgery or RFA and significantly higher in clinically progressive disease compared to stable disease (5.8±0.3 versus 0.6±0.1, p<0.002). The performance metrics for differentiating stable and progressive disease were sensitivity: 91% and specificity: 91%. The score was robust (reproducibility: Coefficient of Variation<2%).Long-term PPI use (>1yr), did not alter the NETest values, neither did age, sex or ethnicity. The PCR score was significantly (p<0.0005) more accurate than CgA for identifying NETs and was elevated in 91% of NETs when CgA was normal. Conclusions: This study demonstrates that a multi (51)-gene NET panel is both sensitive and specific for detecting NETs and is capable of differentiating clinically stable from progressive disease. The test is robust and significantly more sensitive and specific (accurate) than CgA. The NETest thus facilitates both accurate disease detection, and disease progress thereby permitting assessment of treatment efficacy.

Health-related quality of life (HRQoL) in patients with advanced, nonfunctional, well-differentiated gastrointestinal (GI) or lung neuroendocrine tumors (NET) in the phase 3 RADIANT-4 trial.

2016 ◽  
Vol 34 (15_suppl) ◽  
pp. e15657-e15657 ◽  
Author(s):  
Marianne E. Pavel ◽  
Jonathan R. Strosberg ◽  
Lida Bubuteishvili-Pacaud ◽  
Evgeny Degtyarev ◽  
Maureen Neary ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Neuroendocrine Tumors ◽  
Health Related Quality ◽  
Phase 3 ◽  
Related Quality ◽  
Health Related ◽  
Well Differentiated

scholarly journals Non-functioning neuroendocrine tumors of the common hepatic duct: a case report and literature review

2017 ◽  
Vol 4 (3) ◽  
pp. 1101
Author(s):  
Maroun Abou-Jaoude ◽  
Haydar A. Nasser
Keyword(s):  
Neuroendocrine Tumors ◽  
Epigastric Pain ◽  
Hepatic Duct ◽  
Young Patients ◽  
Pancreatic Head ◽  
Long Term Results ◽  
Common Hepatic Duct ◽  
The Common ◽  
Well Differentiated

Extra-hepatic biliary tree neuroendocrine tumors are not common, accounting for about 0.1 % of all carcinoid tumors. Those affecting the common hepatic duct are very rare and their diagnosis is usually made post-operatively. Poorly differentiated tumors or neuroendocrine carcinomas are commonly seen in elderly, whereas well differentiated tumors, tend to occur in young patients, for whom surgery will lead to good long term results. About 100 cases have been reported in the English medical literature, showing good long term results after surgery for well differentiated (Grades 1 and 2) tumors. Herein, we report a case of an 18-year-old female, complaining from a dull epigastric pain related to a nodule compressing the common hepatic duct. After complete investigation, a laparotomy has been performed and showed a nodular tumor located in the common hepatic duct just above the insertion of the cystic duct with close contact with the pancreatic head distally. En bloc cholecystectomy with bile duct resection was performed and followed by a Roux-en-Y hepatico-jejunostomy. The pathology of the nodule came back to be a neuroendocrine tumor grade 2.

scholarly journals Hepatic Arterial Embolization for the Treatment of Metastatic Neuroendocrine Tumors

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Eric Lee ◽  
H. Leon Pachter ◽  
Umut Sarpel
Keyword(s):  
Liver Metastases ◽  
Neuroendocrine Tumors ◽  
Survival Rates ◽  
Arterial Embolization ◽  
Progression Free Survival ◽  
Term Survival ◽  
Free Survival ◽  
Hepatic Arterial Embolization

Neuroendocrine tumors (NETs) have a high predilection for metastasizing to the liver and can cause severe debilitating symptoms adversely affecting quality of life. Although surgery remains the treatment of choice, many liver metastases are inoperable at presentation. Hepatic arterial embolization procedures take advantage of the arterial supply of NET metastases. The goals of these therapies are twofold: to increase overall survival by stabilizing tumor growth, and to reduce the morbidity in symptomatic patients. Patients treated with hepatic arterial embolization demonstrate longer progression-free survival and have 5-year survival rates of nearly 30%. The safety of repeat embolizations has also been proven in the setting of recurrent symptoms or progression of the disease. Despite not being curative, hepatic arterial embolization should be used in the management of NETs with liver metastases. Long-term survival is not uncommon, making aggressive palliation of symptoms an important component of treatment.

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