scholarly journals Advances in the Diagnosis and Management of Well-Differentiated Neuroendocrine Neoplasms

2020 ◽  
Vol 41 (2) ◽  
pp. 371-403 ◽  
Author(s):  
Johannes Hofland ◽  
Gregory Kaltsas ◽  
Wouter W de Herder
Keyword(s):  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
Tumor Grading ◽  
Diagnostic Biomarkers ◽  
Randomized Controlled ◽  
The Past ◽  
Well Differentiated ◽  
Excessive Secretion ◽  
Evidence Based Guidelines

Abstract Neuroendocrine neoplasms constitute a diverse group of tumors that derive from the sensory and secretory neuroendocrine cells and predominantly arise within the pulmonary and gastrointestinal tracts. The majority of these neoplasms have a well-differentiated grade and are termed neuroendocrine tumors (NETs). This subgroup is characterized by limited proliferation and patients affected by these tumors carry a good to moderate prognosis. A substantial subset of patients presenting with a NET suffer from the consequences of endocrine syndromes as a result of the excessive secretion of amines or peptide hormones, which can impair their quality of life and prognosis. Over the past 15 years, critical developments in tumor grading, diagnostic biomarkers, radionuclide imaging, randomized controlled drug trials, evidence-based guidelines, and superior prognostic outcomes have substantially altered the field of NET care. Here, we review the relevant advances to clinical practice that have significantly upgraded our approach to NET patients, both in diagnostic and in therapeutic options.

scholarly journals Integument: Guidelines for the care of people with spina bifida

2020 ◽  
Vol 13 (4) ◽  
pp. 543-548
Author(s):  
Patricia Beierwaltes ◽  
Sharon Munoz ◽  
Jennifer Wilhelmy
Keyword(s):  
Spina Bifida ◽  
Daily Living ◽  
Wound Care ◽  
Consensus Building ◽  
Existing Problems ◽  
The Past ◽  
Whole Person ◽  
History Of ◽  
Evidence Based Guidelines

PURPOSE: Skin-related issues have a significant impact on health, activities of daily living, and quality of life among people with spina bifida. Data presented by select clinics that participate in the National Spina Bifida Patient Registry reported that 26% of individuals had a history of pressure injuries with 19% having had one in the past year. The spina bifida community lack direct guidelines on prevention of these and other skin related issues. The Integument (skin) Guidelines focus on prevention, not treatment, of existing problems. METHODS: Using a consensus building methodology, the guidelines were written by experts in spina bifida and wound care. RESULTS: The guidelines include age-grouped, evidence-based guidelines written in the context of an understanding of the whole person. They are presented in table format according to the age of the person with spina bifida. CONCLUSION: These guidelines present a standardized approach to prevention of skin-related issues in spina bifida. Discovering what results in successful minimization of skin-related issues with testing of technology or prevention strategies is the next step in protecting this vulnerable population.

The Spectrum of Neuroendocrine Tumors: Histologic Classification, Unique Features and Areas of Overlap

2015 ◽  
pp. 92-103 ◽  
Author(s):  
David S. Klimstra ◽  
Himisha Beltran ◽  
Rogerio Lilenbaum ◽  
Emily Bergsland
Keyword(s):  
Neuroendocrine Tumors ◽  
Prostate Gland ◽  
Neuroendocrine Differentiation ◽  
Large Cell ◽  
Genetic Alterations ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
Platinum Based Chemotherapy ◽  
Well Differentiated ◽  
Grade 3

Neuroendocrine neoplasms are diverse in terms of sites of origin, functional status, and degrees of aggressiveness. This review will introduce some of the common features of neuroendocrine neoplasms and will explore the differences in pathology, classification, biology, and clinical management between tumors of different anatomic sites, specifically, the lung, pancreas, and prostate. Despite sharing neuroendocrine differentiation and histologic evidence of the neuroendocrine phenotype in most organs, well-differentiated neuroendocrine tumors (WD-NETs) and poorly differentiated neuroendocrine carcinomas (PD-NECs) are two very different families of neoplasms. WD-NETs (grade 1 and 2) are relatively indolent (with a natural history that can evolve over many years or decades), closely resemble non-neoplastic neuroendocrine cells, and demonstrate production of neurosecretory proteins, such as chromogranin A. They arise in the lungs and throughout the gastrointestinal tract and pancreas, but WD-NETs of the prostate gland are uncommon. Surgical resection is the mainstay of therapy, but treatment of unresectable disease depends on the site of origin. In contrast, PD-NECs (grade 3, small cell or large cell) of all sites often demonstrate alterations in P53 and Rb, exhibit an aggressive clinical course, and are treated with platinum-based chemotherapy. Only WD-NETs arise in patients with inherited neuroendocrine neoplasia syndromes (e.g., multiple endocrine neoplasia type 1), and some common genetic alterations are site-specific (e.g., TMPRSS2-ERG gene rearrangement in PD-NECs arising in the prostate gland). Advances in our understanding of the molecular basis of NETs should lead to new diagnostic and therapeutic strategies and is an area of active investigation.

scholarly journals Combination of ICSs and LABAs Should Be Used in the Management of Patients with COPD — The Con Argument

2004 ◽  
Vol 11 (3) ◽  
pp. 224-226
Author(s):  
Jeremy Road
Keyword(s):  
Inhaled Corticosteroids ◽  
Forced Expiratory Volume ◽  
Chronic Obstructive ◽  
Obstructive Pulmonary Disease ◽  
Randomized Controlled ◽  
The Past ◽  
Related Quality ◽  
Health Related ◽  
Long Acting

The management of patients with symptomatic chronic obstructive pulmonary disease (COPD) has become more clear in the past several years. New medications have been developed and their efficacy has been evaluated using important outcomes in addition to forced expiratory volume in 1 s (FEV1), such as health-related quality of life (HRQL), frequency of exacerbations and dyspnea scores. I will review five welldesigned, randomized, controlled trials that have advanced our knowledge about the use of inhaled corticosteroids (ICSs), long-acting beta2-agonists (LABAs) and their combination.

scholarly journals Treatment Challenges in Severe Eosinophilic Asthma: Differential Response to Anti-IL-5 and Anti-IL-5R Therapy

2021 ◽  
Vol 22 (8) ◽  
pp. 3969
Author(s):  
Agamemnon Bakakos ◽  
Nikoleta Rovina ◽  
Petros Bakakos
Keyword(s):  
Quality Of Life ◽  
Real Life ◽  
Controlled Trials ◽  
Clinical Effects ◽  
Biological Therapies ◽  
Eosinophilic Asthma ◽  
Severe Eosinophilic Asthma ◽  
Randomized Controlled ◽  
The Past

Severe asthma greatly affects patients’ quality of life. Major advances have occurred in the management of severe eosinophilic asthma the past few years due to the new targeted biological therapies. There are three anti-IL-5 mAbs, mepolizumab, reslizumab and benralizumab. Despite the different mechanism of blocking IL-5 the clinical effects are quite similar as randomized controlled trials and real-life studies have shown. Moreover, there are reports of responding to one after failing to respond to another anti-IL-5 therapy. Accordingly, it is challenging to explore the possible differences in the response to anti-IL-5 treatments. This might help us not only understand possible mechanisms that contribute to the resistance to treatment in this particular asthma endotype, but also to phenotype within severe eosinophilic asthma in order to treat our patients more efficiently.

Correlation of DLL3 expression in gastroenteropancreatic neuroendocrine neoplasms with loss of RB1 and prognostic significance.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. 4611-4611
Author(s):  
Chiara Liverani ◽  
Alberto Bongiovanni ◽  
Laura Mercatali ◽  
Federica Pieri ◽  
Chiara Spadazzi ◽  
...  
Keyword(s):  
Ct Scan ◽  
Clinical Characteristics ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
High Grade ◽  
Cell Lung Carcinoma ◽  
Pet Ct ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Pet Ct Scan

4611 Background: Neuroendocrine neoplasms (NENs) are a rare subgroup of tumors with challenging management due to their unpredictable and heterogeneous behaviour. The identification of clinically useful biomarkers is a top priority need in this disease. The negative notch regulator DLL3 has gained increasing attention in small cell lung carcinoma, large cell neuroendocrine carcinoma and neuroendocrine prostate cancer, confirming the tumor suppressor function of Notch-1 signaling in neuroendocrine cells. Methods: A retrospective immunohistochemical analysis of DLL3, PD-L1 and RB1 was performed on FFPE samples from 43 patients with gastroenteropancreatic (GEP)-NENs and correlated with clinical characteristics. Results: DLL3 was expressed in high-grade (G3) GEP-NENs. The presence of DLL3 was significantly associated with poorly differentiated NEC (77.8% positive tumors), while none of the patients with well-differentiated NET expressed this marker. Expression of DLL3 was correlated with loss of RB1 and negative 68Ga-PET/CT scan. The 85.7% of DLL3- positive tumors showed loss of RB1 expression, while only 1 out of 35 DLL3- negative tumors had RB1 loss. DLL3 was expressed in 75% of patients with negative 68Ga-PET/CT, while only in 25% of patients with positive 68Ga-PET/CT scan. The presence of DLL3 was negatively associated with PFS and OS. Median PFS was 41.9 months in DLL3-negative patients versus 7.9 months in DLL3-positive patients; median OS was 72.9 months in DLL3-negative patients versus 11.7 months in DLL3-positive patients. No correlation was found with DLL3 and PD-L1 expression. The presence of PD-L1 was not associated with any clinical characteristics. Conclusions: DLL3 is expressed in high grade GEP-NENs and is associated with loss of RB1, negative 68Ga-PET/CT scan and unfavourable clinical outcome. The presence of DLL3 discriminates poorly differentiated NEC from well-differentiated NET. DLL3 could represent the ideal prognostic factor to stratify patients with GEP-NENs and a candidate therapeutic target in NEC patients.

scholarly journals Landscape and Future Perspectives of Immunotherapy in Neuroendocrine Neoplasia

Cancers ◽  
2020 ◽  
Vol 12 (4) ◽  
pp. 832 ◽  
Author(s):  
Ilaria Maggio ◽  
Lisa Manuzzi ◽  
Giuseppe Lamberti ◽  
Angela Dalia Ricci ◽  
Nastassja Tober ◽  
...  
Keyword(s):  
Current Knowledge ◽  
Single Agent ◽  
Metastatic Potential ◽  
Neuroendocrine Cells ◽  
Whole Body ◽  
Neuroendocrine Neoplasms ◽  
Neuroendocrine Neoplasia ◽  
Well Differentiated ◽  
Third Line ◽  
Line Setting

Background: Neuroendocrine neoplasms are rare entities consisting of a heterogeneous group of tumors that can originate from neuroendocrine cells present in the whole body. Their different behavior, metastatic potential, and prognosis are highly variable, depending on site of origin, grade of differentiation, and proliferative index. The aim of our work is to summarize the current knowledge of immunotherapy in different neuroendocrine neoplasms and its implication in clinical practice. Results: Several studies evaluated the efficacy and safety of immunotherapy in neuroendocrine neoplasms, in any setting of treatment, alone or in combination. Studies led to approval in neuroendocrine neoplasia of the lung, in combination with chemotherapy as first-line treatment or as a single-agent in a third-line setting, and Merkel cell carcinoma as a single agent. Results in other settings have been disappointing so far. Conclusions: Immunotherapy seems a valid treatment option for high grade, poorly differentiated neoplasms. Future trials should explore the combination of immunotherapy with other agents, such as anti-angiogenic or other immunotherapy agents, in order to evaluate potential efficacy in low and intermediate grades, well differentiated tumors.

scholarly journals Modern view on the classification of neuroendocrine neoplasms of stomach

2021 ◽  
Vol 67 (5) ◽  
pp. 600-605
Author(s):  
Ivan Peregorodiev ◽  
Vagan Bokhian ◽  
Vera Delektorskaya ◽  
Anna Safonova
Keyword(s):  
Proton Pump ◽  
Who Classification ◽  
Tnm Classification ◽  
Term Therapy ◽  
Favourable Result ◽  
Neuroendocrine Neoplasms ◽  
Adequate Treatment ◽  
The Past ◽  
Well Differentiated

Neuroendocrine neoplasms (NEN) of the stomach include a heterogeneous group of neoplasms characterized by different pathogenetic mechanisms, metastatic potential, and prognosis. The incidence of gastric NAN has increased 15-fold over the past few decades. It is necessary to clearly distinguish between well-differentiated neuroendocrine neoplasms (NETs) of the stomach and neuroendocrine cancer (NECs) of the stomach, as well as mixed neuroendocrine-non-neuroendocrine neoplasms. To date, there are three classifications that are used in clinical practice: clinicopathological classification, WHO classification, and UICC TNM classification. It should be noted that over the past five years, these classifications have undergone important changes. In the clinicopathological classification, in addition to the existing three types, a number of authors proposed to add the 4th type (developing as a result of inadequate functioning of the proton pump) and the 5th type (developing in patients who receive long-term therapy with proton pump inhibitors). In the 2019 WHO classification, an important change is the acceptance that well-differentiated NETs can have a high degree of malignancy – Grade III. The main changes in the 8th edition of the TNM classification concerned the differentiation of the M symbol. Thus, to date, the classification of gastric NENs allows us to accurately characterize tumor staging, the pathogenesis underlying their development, and prognosis. They form the basis for choosing an adequate treatment strategy that makes possible to achieve the most favourable result.

scholarly journals Primary Mesenteric Carcinoid Tumor Presenting with Carcinoid Syndrome

2018 ◽  
Vol 12 (2) ◽  
pp. 396-401
Author(s):  
Oluwaseun Shogbesan ◽  
Abdullateef Abdulkareem ◽  
Binu Pappachen ◽  
John Altomare
Keyword(s):  
Carcinoid Tumor ◽  
Primary Tumor ◽  
Carcinoid Syndrome ◽  
The Body ◽  
Carcinoid Tumors ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
Diverse Group ◽  
Poorly Differentiated ◽  
Well Differentiated

Neuroendocrine neoplasms (NENs) are a diverse group of tumors arising throughout the body with a common origin from neuroendocrine cells. Well-differentiated NENs, also known as neuroendocrine tumors (NETs), are generally indolent and are often found incidentally, while poorly differentiated tumors are more aggressive. Carcinoid tumors are NETs arising from the gastrointestinal tract and less commonly from the lungs, thymus, and kidneys. NETs in the mesentery arise from metastasis from primary tumor, and carcinoid syndrome in this setting results from concomitant metastasis to the liver. Primary mesenteric carcinoid tumors are very rare. We present a 64-year-old man with carcinoid syndrome from a mesenteric carcinoid tumor without evidence of liver metastasis or other primary tumor sites.

scholarly journals Somatostatin-Dopamine Chimeric Molecules in Neuroendocrine Neoplasms

2021 ◽  
Vol 10 (3) ◽  
pp. 501
Author(s):  
Maria Celeste Cantone ◽  
Alessandra Dicitore ◽  
Giovanni Vitale
Keyword(s):  
Somatostatin Receptors ◽  
Somatostatin Analogues ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
Magic Bullet ◽  
Comprehensive Overview ◽  
New Class ◽  
Well Differentiated ◽  
Chimeric Molecules

Neuroendocrine neoplasms (NENs) are a widely heterogeneous family of neoplasms arising from neuroendocrine cells, which are interspersed throughout the body. Despite NENs are relatively rare, their incidence and prevalence are constantly increasing probably due to the improvement in earlier diagnosis and patients’ management. When surgery is not curative, particularly for patients with metastatic disease, several medical options are available. Somatostatin analogues (SSA) are the first-line medical therapy for well-differentiated NENs. Interestingly, the heterodimerization of somatostatin receptors (SSTs) with dopamine receptors (DRs) has been discovered in NENs. This phenomenon results in hybrid receptors with enhanced functional activity. On these bases, chimeric molecules embracing somatostatin and dopamine features have been recently developed. The aim of this review is to provide a comprehensive overview of the available preclinical and clinical data regarding chimeric somatostatin-dopamine agonists as a new class of “magic bullet” in the therapy of NENs.

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