-
Child's Nervous System : ChNS :... Sep 2022Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare tumor, first described by the WHO Classification of Central Nervous System Tumors in 2016. The clinical... (Review)
Review
BACKGROUND
Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare tumor, first described by the WHO Classification of Central Nervous System Tumors in 2016. The clinical course is variable. Most tumors have low-grade histological findings although some may have more aggressive features. The goal of this systematic review was to identify prognostic factors for poor overall survival (OS).
MATERIAL AND METHODS
We performed a systematic review using three databases (PubMed, Google Scholar, and Embase) and the following search terms: diffuse leptomeningeal glioneuronal tumor, DLGNT, DLMGNT. Statistical analysis was performed using Statistica 13.3.
RESULTS
We included 34 reports in our review comprising 63 patients, published from 2016 to 2022. The median OS was 19 months (range: 12-51 months). Using multivariable Cox survival analysis, we showed that Ki-67 ≥ 7%, age > 9 years, symptoms of elevated intracranial pressure (ICP) at admission, and the presence of contrast-enhancing intraparenchymal tumor are associated with poor OS. Receiver operating characteristic (ROC) analysis identified Ki-67 ≥ 7% as a significant predictor of poor OS.
CONCLUSIONS
Signs or symptoms of increased ICP with imaging findings of diffuse leptomeningeal enhancement should raise suspicion for DLGNT. In our systematic review, Ki-67 ≥ 7% was the most important prognostic factor for OS in DLGNT. The presence of intraparenchymal tumor with contrast enhancement was thought to represent disease progression and, together with patient age, was associated with poor OS.
Topics: Central Nervous System Neoplasms; Child; Humans; Ki-67 Antigen; Meningeal Neoplasms; Neoplasms, Neuroepithelial; Prognosis
PubMed: 35867118
DOI: 10.1007/s00381-022-05600-w -
The Spine Journal : Official Journal of... Sep 2022Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by growing ossifications of spinal entheses and tendons, which may cause trachea and esophagus... (Review)
Review
BACKGROUND AND CONTEXT
Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by growing ossifications of spinal entheses and tendons, which may cause trachea and esophagus compression when located anteriorly in the cervical spine.
PURPOSE
Our previous systematic review on the epidemiological and clinical knowledge of dysphagia and airway obstruction caused by cervical DISH was updated, with a focus on (surgical) treatment and outcomes.
STUDY DESIGN
A systematic review of the literature was performed.
METHODS
Publications in Medline and EMBASE from July 2010 to June 2021 were searched. Two investigators performed data extraction and study specific quality assessment.
RESULTS
A total of 138 articles (112 case reports and 26 case series) were included, describing 419 patients with dysphagia and/or airway obstruction. The mean age of the patient group was 67.3 years (range: 35-91 years), and 85.4% was male. An evident increase of published cases was observed within the last decade. Surgical treatment was chosen for 66% of patients with the anterolateral approach most commonly used. The total complication rate after surgery was 22.1%, with 12.7% occurring within 1 month after intervention. Improvement of dysphagia was observed in 95.5% of operated patients. After a mean follow-up of 3.7 years (range: 0.4-9.0 years), dysphagia recurred in 12 surgically treated patients (4%), of which five patients had osteophyte regrowth.
CONCLUSIONS
The number of published cases of dysphagia in patients with DISH has doubled in the last decade compared to our previous review. Yet, randomized studies or guidelines on the treatment or prevention on recurrence are lacking. Surgical treatment is effective and has low (major) complication rates. Common trends established across the cases in our study may help improve our understanding and management of dysphagia and airway obstruction in cervical DISH.
Topics: Adult; Aged; Aged, 80 and over; Airway Obstruction; Cervical Vertebrae; Deglutition Disorders; Female; Humans; Hyperostosis, Diffuse Idiopathic Skeletal; Male; Middle Aged; Osteophyte
PubMed: 35283294
DOI: 10.1016/j.spinee.2022.03.002 -
North American Spine Society Journal Mar 2024Subjects with ankylosing spinal disorders, including diffuse idiopathic skeletal hyperostosis (DISH) and ankylosing spondylitis (AS) are more prone to vertebral... (Review)
Review
BACKGROUND
Subjects with ankylosing spinal disorders, including diffuse idiopathic skeletal hyperostosis (DISH) and ankylosing spondylitis (AS) are more prone to vertebral fractures and frequently present with neurological deficit compared to the patients without an ankylosed spine. Moreover, prevalent vertebral fractures are an important predictor for subsequent fracture risk. However, the pooled fracture prevalence for DISH is unknown and less recent for AS. We aimed to systematically investigate the prevalence and risk of vertebral fractures in DISH and AS populations.
METHODS
Publications in Medline and EMBASE were searched from January 1980 until July 2023 for cohort studies reporting vertebral fractures in AS and DISH. Data on prevalence were pooled with random effects modeling after double arcsine transformation. Heterogeneity was assessed with I statistics and we performed subgroup analysis and meta-regression to explore sources of heterogeneity.
RESULTS
We included 7 studies on DISH (n = 1,193, total fractures = 231) with a pooled vertebral fracture prevalence of 22.6% (95%CI: 13.4%-33.4%). For AS, 26 studies were included (n = 2,875, total fractures = 460) with a pooled vertebral fracture prevalence of 15.2% (95%CI: 11.6%-19.1%). In general, fracture prevalence for AS remained similar for several study-level and clinically relevant characteristics, including study design, diagnostic criteria, spine level, and patient characteristics in subgroup analysis. AS publications from 2010 to 2020 showed higher fracture prevalence compared to 1990 to 2010 (18.6% vs. 11.6%). Fractures in DISH were most common at the thoracolumbar junction, whereas for AS, the most common location was the mid-thoracic spine.
CONCLUSIONS
Vertebral fractures are prevalent in AS and DISH populations. Differences in fracture distribution along the spinal axis exist between the 2 disorders. Additional longitudinal studies are needed for incident fracture assessment in patients with ankylosing spinal disorders.
PubMed: 38370336
DOI: 10.1016/j.xnsj.2024.100312 -
Thoracic Surgery Clinics Nov 2020Despite multiple diagnostic toolkits, the diagnosis of diffuse malignant pleural mesothelioma relies primarily on proper histologic assessment. The definitive diagnosis...
Despite multiple diagnostic toolkits, the diagnosis of diffuse malignant pleural mesothelioma relies primarily on proper histologic assessment. The definitive diagnosis of diffuse malignant pleural mesothelioma is based on the pathologic assessment of tumor tissue, which can be obtained from core biopsy sampling, pleurectomy, or other more extensive resections, such as extrapleural pneumonectomy. Given its rarity and overlapping microscopic features with other conditions, the histologic diagnosis of diffuse malignant pleural mesothelioma is challenging. This review discusses the pathologic features and the differential diagnosis of diffuse malignant pleural mesothelioma, including select diagnostic pitfalls.
Topics: Biopsy; Diagnosis, Differential; Humans; Mesothelioma, Malignant; Pleural Neoplasms; Thoracic Surgical Procedures
PubMed: 33012427
DOI: 10.1016/j.thorsurg.2020.08.007 -
Journal of Neuroimaging : Official... Jan 2022Diffuse hemispheric gliomas, H3 G34-mutant (DHGs-G34m), are newly recognized malignant brain tumors characterized by histone gene mutations. However, the neuroradiologic... (Review)
Review
BACKGROUND AND PURPOSE
Diffuse hemispheric gliomas, H3 G34-mutant (DHGs-G34m), are newly recognized malignant brain tumors characterized by histone gene mutations. However, the neuroradiologic characteristics of these tumors require elucidation. We reviewed the demographic, clinical, and neuroradiological features of DHGs-G34m.
METHODS
Data were extracted using a database search in MEDLINE, SCOPUS, and Google Scholar in June 2021. Studies assessing pathologically proven DHGs-G34m with each patient's information and neuroradiological findings were included. After screening and reviewing 332 abstracts, 12 articles including 56 cases met the criteria. We also added the findings for three patients evaluated in our hospital. Two board-certified radiologists reviewed all demographic, clinical, and neuroradiological findings of each study. One board-certified pathologist reviewed all pathological data of each study. Kaplan-Meier analyses with log-rank tests were performed to compare the survival between patients with different tumor margin characteristics (well-delineated and ill-defined).
RESULTS
The median patient age at diagnosis was 19 years (range, 6-66 years), and 31/59 patients (52.5%) were men. Supratentorial tumors were observed in all patients (59/59, 100%). Frequent contact with leptomeninges (92.3%) and ependymal regions (87.5%) was observed. The 1- and 2-year survival rates after initial surgery were 66.7% and 40.0%, respectively. DHGs-G34m with ill-defined and well-delineated margins showed significant differences in survival (p = .04).
CONCLUSIONS
DHGs-G34m occur most often in the supratentorial regions of adolescents. Prognosis varies among patients. Evaluation of tumor margins may provide prognostic value.
Topics: Adolescent; Brain Neoplasms; Glioma; Histones; Humans; Male; Mutation; Neuroimaging
PubMed: 34632671
DOI: 10.1111/jon.12939 -
Cancers Jun 2021Adult patients with Hodgkin lymphoma (HL) and diffuse large B-cell lymphoma (DLBCL) have prolonged survival but face the risk of treatment-induced impaired fertility.... (Review)
Review
Male and Female Fertility: Prevention and Monitoring Hodgkin' Lymphoma and Diffuse Large B-Cell Lymphoma Adult Survivors. A Systematic Review by the Fondazione Italiana Linfomi.
BACKGROUND
Adult patients with Hodgkin lymphoma (HL) and diffuse large B-cell lymphoma (DLBCL) have prolonged survival but face the risk of treatment-induced impaired fertility. This systematic review, conducted by Fondazione Italiana Linfomi (FIL) researchers, aims to evaluate the incidence of treatment-related infertility, fertility preservation options, fertility assessment measures, and the optimal interval between the end of treatment and conception.
METHODS
MEDLINE, the Cochrane Library, and EMBASE were systematically searched up to September 2020 for published cohort, case-control, and cross-sectional studies on fertility issues.
RESULTS
Forty-five eligible studies were identified. Gonadotoxicity was related to sex, type and dosage of treatment, and, in females, to age. After receiving alkylating-agent-containing regimens, less than 30% of males recovered spermatogenesis, and 45% of females ≥30 years in age retained regular menstrual cycles. Sperm cryopreservation was offered to the majority of patients; sperm utilization resulted in a 33-61% pregnancy rate. After ovarian tissue transplantation, the spontaneous pregnancy and live birth rates were 38% and 23%; after IVF, the live birth rate was 38.4%. No data could be extracted on the utilization rate of cryopreserved mature oocytes. The results of studies on GnRH analogs are controversial; therefore, their use should not be considered an alternative to established cryopreservation techniques. Sperm count, FSH, and inhibin-B levels were appropriate measures to investigate male fertility; serum AMH levels and antral follicle count were the most appropriate markers for ovarian reserve. No data could be found regarding the optimal interval between the end of treatment and conception.
CONCLUSIONS
The risk of infertility should be discussed with adult lymphoma patients at the time of diagnosis, and fertility preservation options should be proposed before first-line treatment with alkylating-agent-containing regimens.
PubMed: 34207634
DOI: 10.3390/cancers13122881 -
Neurosurgical Review Jun 2023Diffuse gliomas significantly affect patients' daily lives. Because of the high risk of recurrence and anaplasic transformation, repeated surgery can be proposed in... (Meta-Analysis)
Meta-Analysis Review
Diffuse gliomas significantly affect patients' daily lives. Because of the high risk of recurrence and anaplasic transformation, repeated surgery can be proposed in awake condition to prolongs overall survival by limiting and reducing residual tumour volume. However, oncological interest alone is no longer sufficient due to the consequent increase in median survival, and quality of life is becoming an important issue in clinical decision-making. This systematic review focuses on the effects of repeated surgery in awake condition on the quality of life of adults with diffuse glioma through three parameters: return to work, presence of postoperative neurocognitive disorders, and occurrence of epileptic seizures. A systematic review of the last 20 years was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) standards. Summarized data from selected studies were processed quantitatively, using a meta-analysis process, with the Review Manager 5.4 software. Five databases (PubMed, Web of Science, Science Direct, Dimensions, and Embase) were used. Fifteen articles were selected for qualitative analysis and 11 for meta-analysis. One hundred and fifty-one patients (85%) returned to an active socio-professional life after repeated surgery, and 78 (41%) presented neurocognitive disorders in the immediate postoperative period, only 3% (n = 4) of them suffering from permanent disorders. One hundred and forty-nine (78%) participants were free of epileptic seizure after repeated surgery. This systematic review of the literature highlights the benefit of repeated surgery on the quality of life of patients with adult diffuse glioma.
Topics: Adult; Humans; Quality of Life; Brain Neoplasms; Wakefulness; Glioma; Seizures
PubMed: 37382692
DOI: 10.1007/s10143-023-02073-6 -
Cancers Jul 2021The continuously improving treatment outcome for classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL) over the last 25 years has led to a high... (Review)
Review
Late Neurological and Cognitive Sequelae and Long-Term Monitoring of Classical Hodgkin Lymphoma and Diffuse Large B-Cell Lymphoma Survivors: A Systematic Review by the Fondazione Italiana Linfomi.
BACKGROUND
The continuously improving treatment outcome for classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL) over the last 25 years has led to a high number of long-term survivors. The impact of treatment, however, can sometimes be dramatic and long-lasting. Focusing on peripheral neuropathy (PN), cognitive impairment, fatigue, anxiety, and depression, researchers of the Fondazione Italiana Linfomi conducted a systematic review of the literature to collect the available data on sequelae incidence as well as evidence of follow-up strategies for long-term cHL and DLBCL survivors.
METHODS
The review was carried out under the methodological supervision of the Istituto di Ricerche Farmacologiche "Mario Negri", Milan, Italy. The literature search was conducted on three databases (MEDLINE, Embase, and the Cochrane Library) updated to November 2019. The selection process and data extraction were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
RESULTS
A total of 2236 abstracts were screened, 247 full texts were analyzed, and 35 papers were included in the final analysis. Fatigue was the most extensively studied among neuropsychological sequelae, with a mean prevalence among cHL survivors of 10-43%. Although many of the papers showed an increased incidence of PN, cognitive impairment, and anxiety and depression in long-term cHL and DLBCL survivors, no definite conclusions can be drawn because of the methodological limitations of the analyzed studies. No data on monitoring and follow-up strategies of PN and other neuropsychological sequelae were highlighted.
CONCLUSIONS
Based on our findings, future studies in this setting should include well-defined study populations and have a longitudinal trial design to assess the outcomes of interest over time, thus as to structure follow-up programs that can be translated into daily practice.
PubMed: 34298616
DOI: 10.3390/cancers13143401 -
Cancers Dec 2021Cardiotoxicity represents the most frequent cause with higher morbidity and mortality among long-term sequelae affecting classical Hodgkin lymphoma (cHL) and diffuse... (Review)
Review
Late Cardiological Sequelae and Long-Term Monitoring in Classical Hodgkin Lymphoma and Diffuse Large B-Cell Lymphoma Survivors: A Systematic Review by the Fondazione Italiana Linfomi.
Cardiotoxicity represents the most frequent cause with higher morbidity and mortality among long-term sequelae affecting classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL) patients. The multidisciplinary team of Fondazione Italiana Linfomi (FIL) researchers, with the methodological guide of Istituto di Ricerche Farmacologiche "Mario Negri", conducted a systematic review of the literature (PubMed, EMBASE, Cochrane database) according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, in order to analyze the following aspects of cHL and DLBCL survivorship: (i) incidence of cardiovascular disease (CVD); (ii) risk of long-term CVD with the use of less cardiotoxic therapies (reduced-field radiotherapy and liposomal doxorubicin); and (iii) preferable cardiovascular monitoring for left ventricular (LV) dysfunction, coronary heart disease (CHD) and valvular disease (VHD). After the screening of 659 abstracts and related 113 full-text papers, 23 publications were eligible for data extraction and included in the final sample. There was an increased risk for CVD in cHL survivors of 3.6 for myocardial infarction and 4.9 for congestive heart failure (CHF) in comparison to the general population; the risk increased over the years of follow-up. In addition, DLBCL patients presented a 29% increased risk for CHF. New radiotherapy techniques suggested reduced risk of late CVD, but only dosimetric studies were available. The optimal monitoring of LV function by 2D-STE echocardiography should be structured according to individual CV risk, mainly considering as risk factors a cumulative doxorubicine dose >250 mg per square meter (m) and mediastinal radiotherapy >30 Gy, age at treatment <25 years and age at evaluation >60 years, evaluating LV ejection fraction, global longitudinal strain, and global circumferential strain. The evaluation for asymptomatic CHD should be offered starting from the 10th year after mediastinal RT, considering ECG, stress echo, or coronary artery calcium (CAC) score. Given the suggested increased risks of cardiovascular outcomes in lymphoma survivors compared to the general population, tailored screening and prevention programs may be warranted to offset the future burden of disease.
PubMed: 35008222
DOI: 10.3390/cancers14010061 -
Cancers Mar 2022Overall survival after lymphoma has improved in recent years, but the high prevalence of late treatment-related sequelae has been observed as a counterpart. (Review)
Review
Late Endocrine and Metabolic Sequelae and Long-Term Monitoring of Classical Hodgkin Lymphoma and Diffuse Large B-Cell Lymphoma Survivors: A Systematic Review by the Fondazione Italiana Linfomi.
BACKGROUND
Overall survival after lymphoma has improved in recent years, but the high prevalence of late treatment-related sequelae has been observed as a counterpart.
METHOD
In this systematic review, FIL researchers aimed to: (i) estimate the incidence or prevalence of late endocrine-metabolic sequelae, (ii) evaluate the effects of modern therapeutic approaches on incidence or prevalence of late endocrine-metabolic sequelae, and (iii) determine whether there is evidence of follow-up schemes for their screening/early diagnosis in the subset of long-term classical Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL) survivors treated at adult age. The MEDLINE, Embase and the Cochrane Library databases were searched for relevant articles published up to October, 2020. The study selection process was conducted by three independent reviewers and was reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A risk of bias assessment was performed using the Cochrane tool for randomized trials and the Newcastle-Ottawa Scale for observational studies.
RESULTS
In the final analysis, eight studies were included, four of which focused on thyroid disease, two on gonadal dysfunction, one on bone disease and one on metabolic syndrome. Hypothyroidism was reported in up to 60% of adult cHL survivors and was frequently recorded even with modern radiotherapy approaches. Menopause occurred in 52-72% of women after chemotherapy. An 86% reduction in vertebral density was reported following R-CHOP-like chemotherapy. Sarcopenia and metabolic syndrome were reported in 37.9% and 60% of patients, respectively. No validated screening protocols were found for the early diagnosis of long-term treatment-related endocrine and metabolic sequelae, thus the authors finally suggest the execution of screening exams according to the risk category which were identified in the epidemiologic studies.
PubMed: 35326591
DOI: 10.3390/cancers14061439