scholarly journals EPID-13. A POPULATION-BASED ANALYSIS OF CNS TUMOR DIAGNOSES, TREATMENT, AND SURVIVAL IN CONGENITAL AND INFANT AGE GROUPS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii321-iii321
Author(s):  
Muriel Hart ◽  
Amy Mellies ◽  
Alina Beltrami ◽  
Ahmed Gilani ◽  
Adam Green

Abstract BACKGROUND Congenital (<3 months) and infant (3 to 11 months) brain tumors are biologically different from tumors in older children, but epidemiology of these tumors has not been studied comprehensively. Insight into epidemiological differences could help tailor treatment recommendations by age and increase overall survival (OS). METHODS Population-based data from the SEER 18 registries was obtained for 14,493 0-19-year-olds diagnosed with CNS tumors between 1990 and 2015. Incidence, treatment, and survival were analyzed using Chi-square and Kaplan-Meier analyses. RESULTS Between the <3 month, 3–5 month, 6–11 month, and 1–19 year age groups, tumor type distribution differed significantly (p<0.001); high-grade glioma (HGG) was most common in the <3-month-olds, while low-grade glioma (LGG) was most common in the other groups. 5-year OS for all tumors was 36.7% (<3 months), 56.0% (<3–5 months), 63.8% (6–11 months), and 74.7% (1–19 years) (log rank p<0.001). OS by tumor type was worst for <3-month-olds with LGG, medulloblastoma, and other embryonal tumors; OS was worst for 3-5-month-olds with ependymoma, <1-year-olds collectively with atypical teratoid-rhabdoid tumor, and 1-19-year-olds with HGG (log rank p<0.02 for all tumor types). <3-month-olds were least likely to receive any treatment for each tumor type and least likely to undergo surgery for all except HGG. <1-year-olds were far less likely than 1-19-year-olds to undergo radiation for embryonal tumors, as expected, but were also less likely to undergo chemotherapy. CONCLUSIONS Congenital/infant CNS tumors differ pathologically, therapeutically, and prognostically from those in older children. Treatment changes could help address poorer outcomes for these young patients.

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii438-iii438
Author(s):  
Kathleen Dorris ◽  
Jessica Channell ◽  
Ashley Mettetal ◽  
Molly Hemenway ◽  
Natalie Briones ◽  
...  

Abstract BACKGROUND Cannabinoids, including cannabidiol (CBD) and tetrahydrocannabinol (THC), are a class of compounds found in marijuana. Numerous studies in adults have examined cannabinoid use in management of cancer-related symptoms such as nausea, anorexia, and pain. Less is known about the use in the pediatric oncology population. METHODS A prospective observational study has been ongoing since 2016 at Children’s Hospital Colorado to evaluate cannabinoids’ impact using PedsQL™ modules on quality of life of pediatric patients with central nervous system (CNS) tumors who are 2–18 years old. Laboratory assessments of T-cell activity and pharmacokinetics of CBD, THC and associated metabolites are in process. Diaries with exploratory information on cannabinoid use patterns are being collected. RESULTS Thirty-three patients (14:19; male:female) have been enrolled with a median age of 6.4 years (range, 2.9–17.7 years). The most common tumor type in enrolled patients is embryonal tumors (13/33; 39%). Nine (27%) patients have low-grade glial/glioneuronal tumors, and eight (24%) had high-grade/diffuse midline gliomas. The remaining patients had ependymoma or craniopharyngioma. The median time on cannabinoids is 9 months. Most (n=20) patients have used oral products with CBD and THC. One patient continues on cannabinoid therapy in follow up. Preliminary immune function analyses identified impaired neutrophil superoxide anion production and chemotaxis in patients taking cannabinoids at early time points on therapy. CONCLUSIONS Families of children with various CNS tumors are pursuing cannabinoid therapy for both antitumor and supportive care purposes. Analysis of the impact of cannabinoids on patients’ quality of life is ongoing.


Author(s):  
Zhenzhen Rao ◽  
Junjie Hua ◽  
Ruotong Li ◽  
Yanhong Fu ◽  
Jie Li ◽  
...  

Recent changes in population-based prevalence for circulatory system diseases (CSDs) remain unreported either nationally or locally for China. Data were from the two-round health service household interview survey of Hunan Province, China, in 2013 and 2018. A Rao–Scott chi-square test was performed to examine prevalence differences across socio-demographic variables. The overall age-standardized prevalence of CSDs increased substantially between 2013 and 2018 for inhabitants aged 20 years and older (14.25% vs. 21.25%; adjusted odds ratio (OR) = 1.59, 95% CI: 1.24–2.04). Hypertensive disease was the most prevalent type of CSD, accounting for 87.24% and 83.83% of all CSDs in 2013 and in 2018, respectively. After controlling for other socio-demographic factors, the prevalence of CSDs was significantly higher in 2018 (adjusted OR = 1.40), urban residents (adjusted OR = 1.43), females (adjusted OR = 1.12) and older age groups (adjusted OR = 5.36 for 50–59 years, 9.51 for 60–69 years, 15.19 for 70–79 years, and 12.90 for 80 years and older) than in 2013, rural residents, males and the youngest age group (20–49 years). The recent increase in the overall age-standardized CSD prevalence and the large prevalence disparities across urban/rural residents, sex and age groups merit the attention of policymakers and researchers. Further prevention efforts are needed to curb the increasing tendency and to reduce the prevalence of disparities across socio-demographic groups.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii386-iii386
Author(s):  
Nune Karapetyan ◽  
Samvel Danielyan ◽  
Gevorg Tamamyan ◽  
Armen Tananyan ◽  
Liana Safaryan ◽  
...  

Abstract BACKGROUND Central nervous system (CNS) tumors are the second most common malignant neoplasms among children worldwide. The current paper aims to analyze the situation in pediatric neuro-oncology in Armenia from the neurosurgical perspective. METHODS We have collected data of pediatric patients with CNS tumors treated in the Neurosurgery department of “Surb Astvasamayr” Medical Center from 01.01.2010 till 01.12.2019. Incidence by gender, age at diagnosis, and histopathology results were calculated. Survival rates were calculated based on the follow-up results performed until 30.12.2019. RESULTS Hospital-based data showed that during the previous 10 years 47 patients with CNS tumors received neurosurgical treatment in the unit, among them 66% were females. 38.3%, 31.9% and 29.8% of diagnosed patients were aged 0–4, 5–9, and 10–18 respectively. In 41 cases, the disease was not disseminated at diagnosis. The most common observed malignancies were low-grade gliomas (21.3%) and embryonal tumors (19.1%), followed by high-grade gliomas (14.9%) and ependymal tumors (8.5%). Follow-up information only for 33 patients is available. From them, 14 are dead and 19 alive. Survival rates in most common groups were 62.5%, 80%, 50%, and 50% respectively. The median follow-up time was 18 months (range 1–113 months). CONCLUSION Similar to the data reported in the literature, low-grade gliomas, and embryonal tumors are the most frequent pediatric CNS tumors in Armenia. On the other hand, the pediatric CNS tumor survival rates are lower compared to those reported in developed countries.


2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi20-vi20
Author(s):  
Margot Lazow ◽  
Andrew Trout ◽  
Christine Fuller ◽  
Jaime Reuss ◽  
Brian Turpin ◽  
...  

Abstract INTRODUCTION 77Lu-DOTATATE, a radionuclide therapy which binds SST2A, has demonstrated efficacy in neuroendocrine tumors and evidence of CNS penetration, supporting potential expansion within pediatric neuro-oncology. Understanding the prevalence of SST2A expression across pediatric CNS tumors is essential to identify patients who may benefit from somatostatin receptor-targeted therapy and to further elucidate the oncogenic role of SST2A. METHODS SST2A immunohistochemistry (IHC) was performed on tumor specimens and interpreted by two experienced pathologists (blinded), utilizing semi-quantitative scoring of membranous expression within viable tumor. Immunoreactive cell percentage was visually scored as 0 (none), 1 (< 10%), 2 (10-50%), 3 (51-80%), or 4 (>80%). Staining intensity was scored as 0 (none), 1 (weak), 2 (moderate), or 3 (strong). Combined scores for each specimen were calculated by multiplying percent immunoreactivity and staining intensity values (range=0-12). RESULTS A total of 117 tumor samples from 113 patients were analyzed. Significant differences in SST2A IHC scores were observed across histopathologic diagnoses, with consistently high scores in medulloblastoma (mean±SD=7.6±3.6 [n=36]) and meningioma (5.7±3.4 [n=15]), compared to minimal or absent expression in ATRT (0.3±0.6 [n=3]), ETMR (1.0±0 [n=3]), ependymoma (grades I-III; 0.2±0.7 [n=26]), and high-grade glioma (grades III-IV; 0.4±0.7 [n=22]). Pineoblastoma (3.8±1.5 [n=4]) and other embryonal tumors (2.3±3.8 [n=8]) exhibited intermediate, variable expression. Among expressors, there was no association between SST2A IHC score and patient age, sex, presence of metastases, likelihood of relapse, or prior treatment. In a subset of paired primary and recurrent specimens from 3 patients, SST2A IHC scores remained largely unchanged. Among medulloblastomas, SST2A IHC scores were higher in non-SHH (8.6±3.2) than SHH (5.0±3.3) molecular subgroups (p=0.033). CONCLUSIONS High membranous SST2A expression was demonstrated in medulloblastoma, meningioma, and some rarer embryonal tumors with potential diagnostic, biologic, and therapeutic implications. Somatostatin receptor-targeted therapy such as 177Lu-DOTATATE deserves further investigation in these highly SST2A-expressing pediatric CNS tumors.


Author(s):  
Yun Sun ◽  
Zhi-yong Xiong ◽  
Peng-fei Yan ◽  
Liang-lei Jiang ◽  
Chuan-sheng Nie ◽  
...  

We evaluated characteristics and different prognostic factors for survival in age-stratified high-grade glioma in a US cohort. Eligible patients were identified in the Surveillance, Epidemiology, and End Results (SEER) registries and stratified into 3 age groups: 20-39 years old (1,043 patients), 40-59 years old (4,503 patients), and >60 years old (5,045 patients). Overall and cancer-related survival data were obtained. Cox models were built to analyze the outcomes and risk factors. It showed that race was a prognostic factor for survival in patients 40 to 59 years old and in patients ≥60 years old. Partial resection was associated with lower overall survival and cause-specific survival in all age groups (overall survival: 20-39 yr: HR=6.41; 40-59 yr: HR=4.84; >60 yr: HR=5.06; cause-specific survival: 20-39 yr: HR=5.87; 40-59 yr: HR=4.01; >60 yr: HR=3.36). The study highlights that, while some prognostic factors are universal, others are age-dependent. The effectiveness of treatment approaches differs for patients in different age groups. Results of this study may help to develop personalized treatment protocols for glioma patients of different ages.


Cancers ◽  
2020 ◽  
Vol 12 (6) ◽  
pp. 1426
Author(s):  
Claudia Santoro ◽  
Stefania Picariello ◽  
Federica Palladino ◽  
Pietro Spennato ◽  
Daniela Melis ◽  
...  

The natural history of non-optic central nervous system (CNS) tumors in neurofibromatosis type 1 (NF1) is largely unknown. Here, we describe prevalence, clinical presentation, treatment, and outcome of 49 non-optic CNS tumors observed in 35 pediatric patients (0–18 years). Patient- and tumor-related data were recorded. Overall survival (OS) and progression-free survival (PFS) were evaluated. Eighteen patients (51%) harbored an optic pathway glioma (OPG) and eight (23%) had multiple non-optic CNS lesions. The majority of lesions (37/49) were managed with a wait-and-see strategy, with one regression and five reductions observed. Twenty-one lesions (42.9%) required surgical treatment. Five-year OS was 85.3%. Twenty-four patients progressed with a 5-year PFS of 41.4%. Patients with multiple low-grade gliomas progressed earlier and had a lower 5-year PFS than those with one lesion only (14.3% vs. 57.9%), irrespective of OPG co-presence. Non-optic CNS tumors are common in young patients with NF1. Neither age and symptoms at diagnosis nor tumor location influenced time to progression in our series. Patients with multiple lesions tended to have a lower age at onset and to progress earlier, but with a good OS.


2015 ◽  
Vol 33 (3_suppl) ◽  
pp. 355-355
Author(s):  
Oxana V. Makarova-Rusher ◽  
Susanna Varkey Ulahannan ◽  
Austin G. Duffy ◽  
Tim F. Greten ◽  
Sean Altekruse

355 Background: Transplant, resection, and ablation are potentially curative treatments for hepatocellular carcinoma (HCC) with limited outcome data in young-old (65-74) and older (≥75) patients. Methods: We evaluated curative treatment and relative survival (RS) outcomes in patients with HCC in 3 age groups (<65 years, 65-74 years, and ≥75 years). Patients with HCC diagnostic codes (histology 8,170-8,175, morphology C22) were identified in the SEER 18 database from 2000 to 2011. Treatments included curative (transplant, resection, radiofrequency ablation (RFA), and other ablations) and palliative therapies. Primary outcome was 5 year RS. Statistical analysis was performed using Kaplan-Meier and Chi-Square tests. Results: We identified 29,654 cases. The mean age was 62 years with almost 40% of HCC cases in patients over 65 years old. Potentially curable, localized stage rates were similar in all age groups, 46%, 48% and 46%, respectively. As a result of less resection and rare transplant use, fewer cases underwent curative treatments in the group 75 years and older in comparison to all other age groups (15% vs. 27%, p = 0.001). Five-year RS in all 3 age groups (<65 years, 65-74 years, and ≥75 years) was better after resection relative to RFA (47% vs. 35% p<0.0001, 44% vs. 37%, p=0.0093, and 43% vs. 28% p=0.0002). The highest survival was seen after liver transplant. Interestingly, among transplanted patients with HCC, 13% were 65-75 years old. Five-year RS was similar in transplanted patients 65-75 vs. those under 65 (76% vs 74% p=0.65). Conclusions: The use of curative treatments for HCC significantly decreases with age, yet there are clear survival benefits in elderly patients receiving such. Even when considering transplant, the data shows that outcome is as good in elderly patients as in younger patients. The benefit of hepatic resection appears to be superior compared to RFA in all age groups, in our analysis. [Table: see text]


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii320-iii320
Author(s):  
Clare Lambert ◽  
Ran Goldman ◽  
Douglas Cochrane ◽  
Anita Dahiya ◽  
Heidi Mah ◽  
...  

Abstract PURPOSE Delay in diagnosis of central nervous system (CNS) tumors in children is well documented. The aims of this study were to characterize the symptomatology of CNS tumors and the time to diagnosis in a large pediatric hospital in Canada. METHODS Retrospective chart review of children diagnosed with a CNS tumor between 2000 and 2016 in Vancouver, British Columbia, Canada was performed. Data collected included demographics, symptomatology, tumor type, age at diagnosis, known visits to healthcare professionals, neuroimaging, therapy and post treatment relapse or progression. RESULTS 148 children with complete medical records were reviewed. The average age at diagnosis was 87.8 months (standard deviation (SD) = 59.7; median = 72). 50.7% of patients had posterior fossa tumors and 49.3% had supratentorial tumors. 30% of patients were diagnosed after a single visit to a health care provider. 7.7% of children needed more than 4 visits. Median total time to diagnosis (PSI) was 62 days (range = 0-2047 days). The longest prediagnostic interval was first symptom onset to first healthcare provider visit (PSI1, median 37 days). Patients with posterior fossa tumors, presence of metastases, and symptoms of ataxia and paresis were associated with shorter PSI. CONCLUSIONS CNS tumors in children continue to pose a diagnostic challenge with significant variability in time to diagnosis. Our population-based study found that median time from symptoms to seeking medical advice by parents was over a month. It is essential to uncover the reasons for delay and address them where possible.


2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i42-i42
Author(s):  
Sébastien Perreault ◽  
François Doz ◽  
Birgit Geoerger ◽  
Karsten Nysom ◽  
Ingrid Øra ◽  
...  

Abstract Background NTRK gene fusions are oncogenic drivers in various CNS and non-CNS tumors. Larotrectinib is a highly selective TRK inhibitor approved to treat patients with TRK fusion cancer, with an objective response rate (ORR) of 78% across multiple non-CNS cancers (McDermott et al, ESMO 2020). We report updated data on pediatric patients with TRK fusion-positive primary CNS tumors. Methods Patients aged &lt;18 years with primary CNS tumors harboring an NTRK gene fusion enrolled in two clinical trials (NCT02637687, NCT02576431) were identified. Larotrectinib was administered until disease progression, withdrawal, or unacceptable toxicity. Response was investigator assessed. Results By July 2020, 26 pediatric patients with TRK fusion-positive CNS tumors were treated. Tumor histologic subtypes included high-grade glioma (n=13), low-grade glioma (n=7), glioneuronal tumor (n=2), neuroepithelial tumor (n=2), CNS neuroblastoma (n=1), and small round blue cell tumor (n=1). Median age was 7.0 years (range 1.3–16.7). The ORR was 38% (95% CI 20–59%): 3 complete responses, 7 partial responses (including 2 pending confirmation), 14 stable disease, and 2 progressive disease. The ORR in patients with high-grade glioma was 38% (95% CI 14–68%). Nineteen of 21 patients (90%) with measurable disease had tumor shrinkage. The 24-week disease control rate was 77% (95% CI 56–91%). Median duration of response (DoR), PFS and overall survival (OS) were not reached. The 12-month rates for DoR, PFS and OS were 75%, 65%, and 86%, respectively. Duration of treatment ranged from 1.2 to 31.3+ months. Treatment-related adverse events were reported for 15 patients (58%) and were Grade 3–4 in 3 patients (12%), with no discontinuations related to larotrectinib. Conclusions In pediatric patients with TRK fusion-positive CNS tumors, larotrectinib demonstrated durable responses, high disease control rate, and good tolerability. These results support testing for NTRK gene fusions in pediatric patients with CNS tumors.


2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Bas Weenink ◽  
Kaspar Draaisma ◽  
Han Z. Ooi ◽  
Johan M. Kros ◽  
Peter A. E. Sillevis Smitt ◽  
...  

Abstract In multiple tumor types, prediction of response to immune therapies relates to the presence, distribution and activation state of tumor infiltrating lymphocytes (TILs). Although such therapies are, to date, unsuccessful in gliomas, little is known on the immune contexture of TILs in these tumors. We assessed whether low and high-grade glioma (LGG and HGG, grade II and IV respectively) differ with respect to number, location and tumor reactivity of TILs; as well as expression of molecules involved in the trafficking and activation of T cells. Intra-tumoral CD8 T cells were quantified by flow cytometry (LGG: n = 12; HGG: n = 8) and immunofluorescence (LGG: n = 28; HGG: n = 28). Neoantigen load and expression of Cancer Germline Antigens (CGAs) were assessed using whole exome sequencing and RNA-seq. TIL-derived DNA was sequenced and the variable domain of the TCRβ chain was classified according to IMGT nomenclature. QPCR was used to determine expression of T cell-related genes. CD8 T cell numbers were significantly lower in LGG and, in contrast to HGG, mainly remained in close vicinity to blood vessels. This was accompanied by lower expression of chemo-attractants CXCL9, CXCL10 and adhesion molecule ICAM1. We did not observe a difference in the number of expressed neoantigens or CGAs, nor in diversity of TCR-Vβ gene usage. In summary, LGG have lower numbers of intra-tumoral CD8 T cells compared to HGG, potentially linked to decreased T cell trafficking. We have found no evidence for distinct tumor reactivity of T cells in either tumor type. The near absence of TILs in LGG suggest that, at present, checkpoint inhibitors are unlikely to have clinical efficacy in this tumor type.


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