Neuro-oncology*
Date/Time: Monday, September 11, 2023 - 4:15 PM – 5:45 PM
Track: Special Interest Group (SIG) Session
Room: Franklin Hall 4 (4th Floor)
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Description:
Session Evaluation Form: https://myana.org/form/ana2023-session-evaluation-nonco
Chair: Kevin Elmore, MD
Co-Chair: Douglas Ney, MD, FANA
This conference will address special populations within neuro-oncology including underserved populations, women, and children with brain tumors. Despite considerable advancements in our understanding of the molecular basis of neuro-oncologic diseases, health-disparate populations remain affected by limitations in the access to high-quality care. For underserved populations, including minorities and women with brain tumors, barriers in obtaining equitable care results in increased morbidity and mortality and under-representation in clinical trials. Furthermore, women experience particular health issues including reproductive concerns and hormone-dependent tumors, with gaps in understanding in these tumors and their treatments on female sexuality. Lastly, the unique molecular profiles of pediatric brain tumors will be emphasized, namely the influences autophagy on pediatric brain tumor growth. Better understanding the mechanisms of autophagy and manipulation of these pathways be leveraged to improve responses to anti-cancer therapies. This session aims to educate participants on the challenges faced and identify opportunities to improve outcomes within these unique populations.
Learning Objectives:
- Recognizing disparities in health care access and outcomes within under-represented populations.
- Addressing women’s reproductive health and hormone-dependent tumors.
- Understanding the role of autophagy in cancer and how manipulating autophagy may improve brain tumor therapies.
The Neuro-Oncology of Women (NOW): Neuro-Oncology issues across her lifespan
Speaker: NaTosha Gatson MD, PhD, FAAN
The study of the neurobiological and psychosocial impact of brain tumors in women, paired with the academic movement to encourage female focused clinical and translational brain tumor research, has been termed, “Neuro-Oncology of Women (NOW).” While females comprise 60% of adult brain and spinal cord tumors, guidelines that address women’s issues in neuro-oncology are lacking. This presentation sheds light on common women’s issues in neuro-oncology. Neuro-oncology providers are often faced with patient questions about fertility, pregnancy maintenance or prevention, sex and sexuality, and the hormonal impact on tumors over their life-span. These questions often go unanswered or are only done so anecdotally due to the paucity of evidence in this space. These remain important gap-issues that are addressed as part of NOW and greater attention should be given to the subfield.
Autophagy and Targeted Therapy in Brain Tumors
Speaker: Jean M. Mulcahy Levy, MD
Autophagy manipulation in tumors with molecularly-defined mutations has the potential to advance the field of treating RAF pathway-altered CNS tumors. BRAFV600E-positive gliomas exhibit an increase in autophagy and an addiction to this survival pathway. This provides the initial biomarker for patients who could benefit from blocking autophagy clinically. Autophagy inhibition can overcome mechanistically distinct forms of resistance to RAFi, providing a new way to circumvent a major limitation of these drugs.
Underserved Populations in Neuro-Oncology
Speaker: Shawn Hervey-Jumper, MD
The NIH Revitalization Act, implemented 29 years ago, set to improve the representation of women and minorities in clinical trials. In this abstract, we review progress made in all phase therapeutic clinical trials for neuro-epithelial CNS tumors stratified by demographic-specific age-adjusted disease incidence and mortality. Additionally, we identify workforce characteristics associated with clinical trials meeting established accrual benchmarks.
Registry study of published clinical trials for World Health Organization defined neuro-epithelial CNS tumors between January 2000 and December 2019. Study participants were obtained from PubMed and ClinicalTrials.gov. Population-based data originated from the CBTRUS for incidence analyses. SEER-18 Incidence-Based Mortality data was used for mortality analysis. Descriptive statistics, Fisher exact, and 2 tests were used for data analysis.
Among 662 published clinical trials representing 49,907 participants, 62.5% of study participants were men and 37.5% were women (P<0.0001) representing a mortality specific over-accrual for men (P=0.001). Whites, Asians, Blacks, and Hispanics represented 91.7%, 1.5%, 2.6%, and 1.7% of trial participants. Compared with mortality, Blacks (47% of expected mortality, P=0.008), Hispanics (17% of expected mortality, P<0.001) and Asians (33% of expected mortality, P<.001) were underrepresented compared with Whites (114% of expected mortality, P<0.001). Clinical trials meeting accrual benchmarks for race included minority authorship.
Following the Revitalization Act, minorities and women remain underrepresented when compared with their demographic-specific incidence and mortality in therapeutic clinical trials for neuroepithelial tumors. Through this session we will prove a framework for clinical trial accrual efforts and offers guidance regarding workforce considerations associated with enrollment of underserved patients.
Receipt of Guideline Concordant-Care for Neurofibromatosis 1 (NF1) in the United States: A National Survey of NF1 Patients and Caregivers
Oral Abstract Presenter: Vanessa Merker, PhD
Background: Recent clinical care guidelines from the AAP and ACMG recommend specific health screenings and education for children and adults with NF1, a rare neurogenetic disorder. We assessed the extent to which these guidelines have been implemented in the U.S. and socio-demographic predictors of receiving guideline-concordant care. Methods:An electronic survey was sent to U.S.-based NF1 patients and parents/caregivers enrolled in the Children’s Tumor Foundation Neurofibromatosis Registry in May 2021. Survey topics included demographics, location and specialty of NF care providers, and self-reported receipt of guideline-concordant clinical care (including blood pressure measurement, skin exam, and scoliosis screening in children and adults; tracking of pubertal development and developmental milestone/school progress in children; and education about family planning and warning signs/symptoms of cancer for adults). We used inverse propensity score reweighting of survey responses to estimate receipt of guideline-concordant care among the entire NF1 registry population and used robust linear regression to look for potential socio-demographic disparities in the total number of age-appropriate recommended health services individuals received. Results: 322 individuals with NF1 responded (4.6% response rate, 160 adults and 162 parent/caregivers, 58% female, 78% white). 53.6% of children and 25.2% of adults received at least 4 out of the 5 recommended health services for their age group. Individuals who visited a specialized NF1 clinic within the past three years (β=0.86, p<0.001 for children; β=0.76, p=0.002 for adults) or who had an appointment with their NF1 care provider in the past year (β=1.62, p<0.001 for children; β=1.23, p<0.001 for adults) received more guideline-concordant evaluations. Among adults, White patients were more likely than Black patients (β=0.95, p=0.027) and patients of other races (β=0.70, p=0.043) to receive guideline-concordant care, as were adults with commercial insurance compared to Medicaid (β=1.096, p=0.011) and adults who saw a neurologist for their NF1 care (β=0.48, p=0.038). Family income, education, urbanicity/rurality, and seeing a geneticist for NF1 care were not significantly related to receipt of guideline-recommended care in children or adults. Conclusions: Specialized NF1 clinics and neurologists were more likely to provide patient-reported, guideline-concordant NF1 care than other providers, but some racial and economic disparities in quality of care persist. A recently funded online intervention to disseminate personalized, evidence-based care recommendations to patients and their local care teams could improve the quality of NF1 care in the U.S.
The Natural History of Neurolymphomatosis
Oral Abstract Presenter: Eric Wong, MD, FANA, FAAN
Purpose: Neurolymphomatosis is a rare lymphoid malignancy of the peripheral nervous system. Patients suffer from debilitating neurological deficits due to lymphoma invasion into one or more peripheral nerves and the natural history of this disease is still poorly understood. Methods: We performed a PubMed search and extracted data on (i) year of publication, (ii) age, (iii) gender (iv), type of lymphoma, (v) nerves involved), (vi) imaging modalities, (vii) treatment received, and (viii) survival times. Kaplan-Meier statistics were used to determine outcome parameters over time and compare prognostic factors. Multiple comparisons were done to determine differences among groups. Results: Our search identified 559 patient cases from 286 published articles. Median age was 61 years (range 2- 92 years) from 326 males, 222 females, and 11 with unspecified gender. Median overall survival (OS) was 12.0 months (range 10.0-15.0) for the entire cohort. Most frequent histologies were (i) diffuse large B-cell lymphoma, (ii) unspecified T-cell lymphoma, and (iii) unspecified B-cell lymphoma, involving the (a) brachial plexus, (b) cranial nerves, and (c) sciatic nerve. None had molecular profiling. There was a progressive lengthening of OS in successive decades, from 0.5 (95%CI 0.0-0.8) to 26.4 (95%CI 18.0- 34.8) months between 1951 and 2022 (r2=0.9432, p<0.001). Time from first treatment to progression also increased from 2.0 (95%CI N/A) to 28.3 (95%CI 23.1-33.5) months (r2=0.6923, p<0.001). However, time from symptom onset to diagnosis remained unchanged during this period (r2=0.6206, p=0.277). Patients were most frequently treated with (i) methotrexate or methotrexate-based combination chemotherapies (n=81), (ii) rituximab alone or with other chemotherapies (n=176), and/or (iii) radiation (n=79). Only 12 received 3 or more regimens. Primary neurolymphomatosis had a better prognosis than secondary neurolymphomatosis, median OS 15.0 (95%CI 11.0-33.0) vs. 10.0 (95%CI 10.0-10.0) months (p=0.016). No OS difference was noted between B- and T-cell disease (p=0.4393), while patients with low-grade B-cell neurolymphomatosis did better than those with Burkitt’s lymphoma (p<0.001). Conclusions: Outcome for patients with neurolymphomatosis has improved over time from advances in drug treatment. But timely diagnosis remains a major problem that needs improvement.