scholarly journals Changing biological behaviour of NETs during the evolution of the disease: progress on progression

2021 ◽  
Vol 28 (5) ◽  
pp. R121-R140
Author(s):  
Krystallenia I Alexandraki ◽  
Ariadni Spyroglou ◽  
Stylianos Kykalos ◽  
Kosmas Daskalakis ◽  
Georgios Kyriakopoulos ◽  
...  

Following improvements in the management and outcome of neuroendocrine neoplasms (NENs) in recent years, we see a subset, particularly of pancreatic NENs, which become more aggressive during the course of the disease. This is reflected by an increase in the Ki-67 labelling index, as a marker of proliferation, which may lead to an occasion of increase in grading, but generally does not appear to be correlated with histologically confirmed dedifferentiation. A systematic review of the literature was performed in PubMed, Cochrane Library, and Embase until May 2020 to identify cases that have behaved in such a manner. We screened 244 articles: only seven studies included cases in their cohort, or in a subset of the cohort studied, with a proven increase in the Ki-67 during follow-up through additional biopsy. In addition to these studies, we have also tried to identify possible pathophysiological mechanisms implicated in advanced NENs, although currently no studies appear to have addressed the mechanisms implicated in the switch to a more aggressive biological phenotype over the course of the disease. Such progression of the disease course may demand a change in the management. Summarising the overall evidence, we suggest that future studies should concentrate on changes in the molecular pathways during disease progression with sequential biopsies in order to shed light on the mechanisms that render a neoplasm more aggressive than its initial phenotype or genotype.

2018 ◽  
Vol 40 (1) ◽  
pp. 3-14 ◽  
Author(s):  
Lydia Morgan ◽  
Yvonne E. Wren

Children’s speech development begins in infancy. The pattern of this development has been explored in studies over a number of years using a range of research methodology and approaches to investigation. A systematic review of the existing literature was carried out to determine the collective contribution of this literature to our understanding of early vocalizations and babbling through the period 9 to 18 months. Eight bibliographic databases were searched as well as the Cochrane library. Thirteen studies were identified for inclusion, which were mostly longitudinal observational case series. The review identified progressive increases in the complexity and volume of infants’ early vocalizations through the period. It also found a broad order of phonological acquisition. Although the studies in this review demonstrated marked individual variation, the review provides indicative patterns of development which can be used as a basis to explore relationships with later speech development in future studies.


2016 ◽  
Vol 89 (3) ◽  
pp. 378-383 ◽  
Author(s):  
Claudia Burz ◽  
Ben Youssef Mohamed Aziz ◽  
Loredana Bălăcescu ◽  
Luminiţa Leluţiu ◽  
Rareş Buiga ◽  
...  

Background and aims. The aim of this study was to investigate the value of serum carcinoembryonic antigen (CEA) and carbohydrate antigen (CA 19-9) correlated with some tissue molecules as predictive markers for recurrence in colon cancer.Methods. A total of 30 patients diagnosed with colon cancer stage II or III who underwent optimal surgery were enrolled in study. Tumor markers CEA and CA 19-9 were determined before surgery. Tumor samples were prepared using tissue microarray kit (TMA) then stained for different cellular markers (Ki 67, HER2, BCL2, CD56, CD4, CD8) and analyzed using Inforatio programme for quantitative determination. All patients received standard adjuvant treatment, which consisted of eight cycles chemotherapy type XELOX. The patients were followed up for 3 years.Results. Upon 3 years follow-up, 67% of patients developed tumor relapse, the most common site of metastasis being the liver. No correlations were observed between either serum or tissue tumor markers and the risk of tumor relapse.Conclusion. Over 50% of patients with colon cancer who had optimal treatment developed metastasis. No statistically significant predictive value for investigated molecules was found. Future studies are needed to confirm the use of molecular markers in monitoring patients with colorectal cancer


2015 ◽  
Vol 23 (1) ◽  
pp. R27-R41 ◽  
Author(s):  
Krystallenia I Alexandraki ◽  
Gregory A Kaltsas ◽  
Simona Grozinsky-Glasberg ◽  
Eleftherios Chatzellis ◽  
Ashley B Grossman

Gastrointestinal neuroendocrine neoplasms (GI-NENs) are increasingly being recognised, while appendiceal NENs (aNENs) currently constitute the third most common GI-NEN. Appendiceal NENs are generally considered to follow an indolent course with the majority being localised at diagnosis. Thus, the initial surgical approach is not that of a planned oncological resection. Due to the localised nature of the disease in the majority of cases, subsequent biochemical and radiological assessment are not routinely recommended. Histopathological criteria (size, mesoappendiceal invasion, Ki-67 proliferation index, neuro- and angio-invasion) are mainly used to identify those patients who are also candidates for a right hemicolectomy. Goblet cell carcinoids are a distinct entity and should be treated as adenocarcinomas. Despite the absence of any substantial prospective data regarding optimal management and follow-up, recent consensus statements and guidelines have been published. The purpose of this review is to overview the published studies on the diagnosis and management of appendiceal NENs and to suggest a possible management protocol.


Respiration ◽  
2021 ◽  
pp. 1-9
Author(s):  
Laura López-López ◽  
Janet Remedios Rodríguez-Torres ◽  
Lawrence Patrick Cahalin ◽  
Irene Cabrera-Martos ◽  
Irene Torres Sánchez ◽  
...  

<b><i>Background:</i></b> The peripheral and central repercussions of Parkinson’s disease (PD) affect the neuromuscular system producing a loss of muscle strength that can influence the respiratory system. Although several studies have examined various respiratory aspects of PD, to the best of our knowledge no study to date has systematically reviewed the existing data. <b><i>Objectives:</i></b> To examine the available literature related to the respiratory impairment in PD patients. <b><i>Methods:</i></b> We used PRISMA guidelines when reporting this review. We searched Pubmed, Cinhal, SciELO, and Cochrane Library, from inception until August 2018. Main variables assessed were forced vital capacity percent predicted (FVC%) and forced expiratory volume in 1 s percent predicted (FEV<sub>1</sub>%) for PD patients. <b><i>Results:</i></b> Six studies were included in this systematic review and meta-analysis. The obtained results concluded that PD patients present poorer pulmonary function when compared to healthy controls. When PD patients were compared between ON and OFF states, the results reviewed are in favour of the ON state. In the meta-analysis performed for FVC% and FEV<sub>1</sub>%, the results fail to find significant differences between PD patients and controls (<i>p</i> = 0.336 and <i>p</i> = 0.281, respectively), and between PD ON and OFF states (<i>p</i> = 0.109 and <i>p</i> = 0.059, respectively). <b><i>Conclusions:</i></b> We conclude that PD patients have impaired respiratory capacities that are related to the PD severity, time since diagnosis, and OFF state. Adequate follow-up of the respiratory function and studies focused on PD phenotypes have to be considered in future studies.


2018 ◽  
Vol 46 (1-2) ◽  
pp. 1-9 ◽  
Author(s):  
Tzu-Chao Lin ◽  
Bing-Yan Zeng ◽  
Yen-Wen Chen ◽  
Meng-Ni Wu ◽  
Tien-Yu Chen ◽  
...  

Background and Purpose: Periodic limb movements of sleep (PLMS) are usually comorbid with hypertension, tachycardia, and coronary arterial diseases, which are also risk factors for cerebrovascular accidents (CVA). However, evidence about the relationship between CVA and PLMS is still weak. The aim of this study was to investigate (1) the prevalence of CVA in patients with PLMS, and (2) the severity of PLMS in patients with or without CVA through a meta-analysis. Methods: The electronic databases of PubMed, Embase, ScienceDirect, ClinicalKey, Cochrane Library, ProQuest, Web of Science, and ClinicalTrials.gov were searched. The inclusion criteria were (1) articles investigating comorbidity between PLMS and CVA, and (2) clinical trials in humans. Results: This meta-analysis included (1) 9,823 patients with PLMS and 9,416 controls from 5 studies to analyze the prevalence of CVA in PLMS, and (2) 158 patients with PLMS with CVA and 88 PLMS controls without CVA from 3 studies to analyze the severity of PLMS with and without CVA. The results showed (1) significantly higher comorbidity rates of CVA in the patients with PLMS than in the controls without PLMS (OR 1.267, p = 0.019), and (2) higher PLM index in the patients with CVA than in the controls (Hedges’ g = 0.860, p = 0.001; means difference: 4.435, p = 0.016). Conclusions: The results revealed (1) a worse severity of PLMS in the patients with CVA, and (2) increased prevalence of CVA in the patients with PLMS. Based on our results, the patients had a higher prevalence of CVA within 8 years of a diagnosis of PLMS compared to those without PLMS by about 1.3-fold. Whether (1) patients with PLMS receiving treatment have a similar incidence of stroke to those without PLMS, and (2) secondary stroke prevention can lower the severity of PLMS or whether those with severe PLMS have a higher risk of stroke is still inconclusive. Future studies investigating the prevalence of CVA in patients with PLMS should use a follow-up period of over 8 years.


2021 ◽  
Vol 11 ◽  
Author(s):  
Franz Sesti ◽  
Tiziana Feola ◽  
Giulia Puliani ◽  
Roberta Centello ◽  
Valentina Di Vito ◽  
...  

BackgroundParagangliomas (PGLs) are neuroendocrine neoplasms arising from chromaffin cells of sympathetic or parasympathetic paraganglia. Systemic therapies have been used only in metastatic PGLs. Antiangiogenic agents, such as sunitinib, could be a viable therapeutic choice in the subgroup of patients with SDH-positive PGLs. We describe the case of a man with Familial Paraganglioma Syndrome type 1 (FPGL) related to a novel mutation in SDHD gene treated with sunitinib. Furthermore, we performed a systematic review of the literature aimed to address the following question: is sunitinib treatment effective in patients with advanced/progressive/metastatic PGL?MethodsWe performed a data search using MEDLINE, Cochrane Library, and Scopus between April 2019 and September 2020. We included studies reporting data on clinical or biological characteristics, or clinical outcomes of patients with PGLs treated with sunitinib.ResultsThe search leaded to the selection of 25 publications. Data from case reports and case series showed that disease control rate (DCR = stable disease + partial response + complete response) was achieved in 34.7% of cases under sunitinib treatment. In 39% of patients DCR was followed by progressive disease (PD) or tumor relapse, 26.1% patients showed PD. Data from clinical trials showed that DCR was 83%, and the median progression free survival was 13.4 months.DiscussionData from the present literature review suggested that sunitinib could be a viable therapeutic option in advanced/progressive/metastatic inoperable PGLs. However, further trials on the efficacy of sunitinib in FPGL and sporadic PGL are needed.


2018 ◽  
Vol 23 (1) ◽  
pp. 26-31
Author(s):  
Julio César Suárez ◽  
Enrique José Herrera ◽  
Alberto Surur ◽  
Ricardo Theaux ◽  
Silvia Zunino ◽  
...  

Objective: to present our 19 years’ experience in the management of oligodendroglioma brain tumors in adults. Materials and Methods: From a series of 505 adult patients with brain tumors operated on from January 1991 to December 2009, 30 cases (5.9%) were histopathologically diagnosed as oligodendrogliomas. Twenty-six of them achieved a good follow-up. Results: from the 26 patients, the age ranged between 23 and 72 years. Histological diagnosis showed pure oligodendrogliomas in 17 patients and 9 cases of oligoastrocytomas. Ki-67 immunohistochemistry was performed in 23 patients: in 16 positivity was less than or equal to 5% and in 7 it was greater than 5%. Chromosomes 1p and 19q were studied in 12 patients with positive co-deletion in 2 and negative in the other 10. Twenty patients were operated on,3 received radiation therapy and chemotherapy, and 1 brachytherapy with Iodine-125 and two were not operated on (because the diagnosis was incidental, the tumor was small and was located in the motor area). Currently, 19 patients are alive (73%) and 7 died (27%). Mean survival time of the deceased patients was 7 years and 2 months and a median of 5 years and 8 months. Conclusion: it is not possible to make statistical analysis because small number of cases, although total removal seems to be the best treatment option, due to our small series of cases, we hope randomized double-blinded future studies will clear up the best choice of diagnosis and treatment. 


Author(s):  
Adam Lee ◽  
Adam Bajinting ◽  
Abby Lunneen ◽  
Colleen M. Fitzpatrick ◽  
Gustavo A. Villalona

AbstractReports of incidental pneumomediastinum in infants secondary to inflicted trauma are limited. A retrospective review of infants with pneumomediastinum and history of inflicted trauma was performed. A comprehensive literature review was performed. Three infants presented with pneumomediastinum associated with inflicted trauma. Mean age was 4.6 weeks. All patients underwent diagnostic studies, as well as a standardized evaluation for nonaccidental trauma. All patients with pneumomediastinum were resolved at follow-up. Review of the literature identified other cases with similar presentations with related oropharyngeal injuries. Spontaneous pneumomediastinum in previously healthy infants may be associated with inflicted injuries. Clinicians should be aware of the possibility of an oropharyngeal perforation related to this presentation.


2015 ◽  
Vol 24 (3) ◽  
pp. 287-292 ◽  
Author(s):  
Petra A. Golovics ◽  
Laszlo Lakatos ◽  
Michael D. Mandel ◽  
Barbara D. Lovasz ◽  
Zsuzsanna Vegh ◽  
...  

Background & Aims: Limited data are available on the hospitalization rates in population-based studies. Since this is a very important outcome measure, the aim of this study was to analyze prospectively if early hospitalization is associated with the later disease course as well as to determine the prevalence and predictors of hospitalization and re-hospitalization in the population-based ulcerative colitis (UC) inception cohort in the Veszprem province database between 2000 and 2012. Methods: Data of 347 incident UC patients diagnosed between January 1, 2000 and December 31, 2010 were analyzed (M/F: 200/147, median age at diagnosis: 36, IQR: 26-50 years, follow-up duration: 7, IQR 4-10 years). Both in- and outpatient records were collected and comprehensively reviewed. Results: Probabilities of first UC-related hospitalization were 28.6%, 53.7% and 66.2% and of first re-hospitalization were 23.7%, 55.8% and 74.6% after 1-, 5- and 10- years of follow-up, respectively. Main UC-related causes for first hospitalization were diagnostic procedures (26.7%), disease activity (22.4%) or UC-related surgery (4.8%), but a significant percentage was unrelated to IBD (44.8%). In Kaplan-Meier and Cox-regression analysis disease extent at diagnosis (HR extensive: 1.79, p=0.02) or at last follow-up (HR: 1.56, p=0.001), need for steroids (HR: 1.98, p<0.001), azathioprine (HR: 1.55, p=0.038) and anti-TNF (HR: 2.28, p<0.001) were associated with the risk of UC-related hospitalization. Early hospitalization was not associated with a specific disease phenotype or outcome; however, 46.2% of all colectomies were performed in the year of diagnosis. Conclusion: Hospitalization and re-hospitalization rates were relatively high in this population-based UC cohort. Early hospitalization was not predictive for the later disease course.


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