Radiation therapy for infants with cancer.

The clinical outcomes for infants with malignant tumors are often worse than older children due to a combination of more biologically aggressive disease in some cases, and increased toxicity-or deintensification of therapies due to concern for toxicity-in others. Especially in infants and very young children, finding the appropriate balance between maximizing treatment efficacy while minimizing toxicity-in particular late side effects-is crucial. We review here the management of malignant tumors in infants and very young children, focusing on central nervous system (CNS) malignancies and rhabdomyosarcoma.

Calidad de vida en niños supervivientes de tumor de sistema nervioso central / Quality of life in child survivors of central nervous system tumor

INTRODUCCIÓN: Los cuidados de calidad de los supervivientes incluyen vigilancia de la recurrencia y de segundos tumores, intervención en el control de síntomas y de necesidades psicosociales y coordinación del cuidado. OBJETIVO: Conocer la calidad de vida en los niños sobrevivientes de cáncer que son vistos en la consulta externa del servicio de Oncología Pediátrica. MATERIAL Y MÉTODO: Estudio descriptivo transversal, realizado en la consulta externa del Servicio de Oncología Pediátrica del Centro Médico Nacional 20 de noviembre ISSSTE, con 16 pacientes supervivientes de Tumor de Sistema Nervioso Central aplicando Inventario de calidad de vida. (PedsQL MT) Resultados: La dimensión menos afectada fue la social con una media de 73.44 seguida de la física tuvo una media de 71.87, con emocional media de 67.19 y la escolar fue la que menos afectada estuvo con media de 68.75. CONCLUSIÓN: Es fundamental que el médico detecte problemas tardíos derivados del tratamiento y de la enfermedad, prevenir complicaciones y secuelas graves, otorgar ayuda al niño y a su familia para integrarse a la sociedad necesarias para enfrentar a la sociedad e integrarse a ella sin dificultades, logrando así una mejor calidad de vida.

INTRODUCTION: Quality care survivors include surveillance of recurrence and second tumors, intervention in the control of symptoms, psychosocial needs and coordination of care. OBJECTIVE: To determine the quality of life in childhood cancer survivors who are seen in outpatient pediatric oncology service. MATERIALS AND METHODS: A cross sectional study was performed in the outpatient clinic of pediatric oncology of National Medical Center "20 de Noviembre" ISSSTE, with 16 surviving patients of central nervous system tumor applying quality of life inventory. (PedsQL TM) RESULTS: The least affected was the social dimension with an average of 73.44 followed by physics which had an average of 71.87, emotional with a mean of 67.19 and school was the least affected with an average of 68.75. CONCLUSIONS: It is essential that the physician detected late problems from treatment and disease, prevent complications and serious sequelae, grant helps the child and family to join the society and integrate it seamlessly, making a better quality of life.

Complete response to therapy: why do primary central nervous system lymphoma patients not return to work?

PURPOSE: Although primary central nervous system lymphomas (PCNSL) represent extremely aggressive brain tumours, high-dose methotrexate in combination with other chemotherapeutic agents has resulted in long-term disease control in a substantial fraction of patients. Advances in treatment efficacy with longer survival resulted in a focus on additional outcome measures such as quality of life (QoL) and neurocognition. Despite recent evidence of return to work as an important aspect of patients' QoL, little is known about occupational reintegration in PCNSL long-term survivors. This study aimed to detect specific characteristics of patients who successfully resumed work after complete response to therapy. METHODS: Patients with ongoing complete response to therapy completed a test battery capturing neurocognition, social integration, QoL and psychological burden. Of 25 patients who had been in regular employment before diagnosis only eight returned to work after treatment (32%). RESULTS: Patients who resumed work rated important aspects of their QoL and social integration as higher and suffered less from symptoms affecting QoL than patients who did not resume work. Also, the subjective confidence in their ability to work was higher in patients who resumed work, but independent predictors of return to work were not found in logistic regression analyses. CONCLUSION: Occupational (re)integration is of clinical relevance in PCNSL patients after complete response to therapy. Due to the small size of our cohort the present results should be considered an exploratory first step. Return to work might be a crucial aspect of QoL and (re)integration into society after cure of PCNSL.

Health-Related Quality of Life and Patient-Reported Outcomes in Radiation Oncology Clinical Trials.

OPINION STATEMENT: The importance of assessing health-related quality of life (HRQoL) and patient-reported outcomes (PROs) is now well recognized as an essential measure when evaluating the effectiveness of new cancer therapies. Quality of life measures provide for a multi-dimensional understanding of the impact of cancer treatment on measures ranging from functional, psychological, and social aspects of a patient's health. Patient-reported outcomes provide for an assessment of physical and functional symptoms that are directly elicited from patients. Collection of PROs and HRQoL data has been shown to not only be feasible but also provide for reliable measures that correlate with established outcomes measures better than clinician-scored toxicities. The importance of HRQoL measures has been emphasized by both patients and clinicians, as well as policy makers and regulatory bodies. Given the benefits associated with measuring HRQoL and PROs in oncology clinical trials, it is increasingly important to establish methods to effectively incorporate PROs and HRQoL measures into routine clinical practice.

Prediagnostic presentations of glioma in primary care: a case-control study.

Aim: This study aimed to assess the prevalence of symptoms glioma patients may present with to the general practitioner, and whether these can be distinguished from patients with other CNS disorders or any other condition. Methods: Glioma patients were matched to CNS patients and 'other controls' using anonymized general practitioner registries. Prevalences were evaluated in the 5 years prior to diagnosis. Result: CNS patients reported significantly more motor symptoms in the period 60-24 months, (p = 0.039). Moreover, <6 months before diagnosis CNS patients differed significantly in mood disorders/fear compared with 'other controls' (p = 0.012) but not glioma patients (p = 0.816). Conclusion: Glioma patients could not be distinguished from both control groups with respect to the number or type of prediagnostic symptoms.

Social attainment in survivors of pediatric central nervous system tumors: a systematic review and meta-analysis from the Children's Oncology Group.

PURPOSE: Improved therapies for pediatric central nervous system (CNS) tumors have increased survival rates; however, many survivors experience significant long-term functional limitations. Survivors of pediatric CNS tumors can experience deficits in social attainment. The aim of this review was to systematically amalgamate findings pertaining to social attainment (i.e., educational attainment, marriage, employment outcomes) in survivors of pediatric CNS tumors. METHODS: PubMed (web-based), PsycINFO (EBSCO), EMBASE (Ovid), and Web of Science (Thomson Reuters) were used to identify articles published between January 2011 and September 2018. Eligible studies reported outcomes for survivors of pediatric CNS tumors diagnosed before age 21 years and > 5 years from diagnosis and/or > 2 years off therapy. All data were independently abstracted by two reviewers. Random-effects meta-analyses were performed using Review Manager 5.0. RESULTS: The search yielded 7021 unique publications. Forty-six were included in the current review. Meta-analyses revealed survivors of CNS tumors were significantly more likely to have completed compulsory education only (OR = 1.87, 95% CI = 1.66, 2.12, p < 0.00001), less likely to be married (OR = 4.70, 95% CI = 3.89, 5.68, p < 0.00001), and more likely to be unemployed (OR = 2.84, 95% CI = 2.62, 3.08, p < 0.00001) compared to non-cancer controls. Cranial radiation therapy, neurocognitive deficits, and younger age at diagnosis were associated with poorer outcomes. Hearing loss and bilateral blindness were also related to poorer outcomes. Sex did not impact social attainment outcomes. CONCLUSIONS: Survivors of pediatric CNS tumors are at elevated risk for poor attainment of key adult social outcomes. IMPLICATIONS FOR CANCER SURVIVORS: There is a critical need to develop interventions to support survivors in becoming independent and productive adults.

Longitudinal cognitive assessment in patients with primary CNS lymphoma treated with induction chemotherapy followed by reduced-dose whole-brain radiotherapy or autologous stem cell transplantation.

INTRODUCTION: The standard treatment for primary central nervous system lymphoma (PCNSL) involves induction methotrexate-based chemotherapy with or without consolidation whole brain radiotherapy (WBRT). As WBRT carries a substantial risk for cognitive impairment, alternative consolidation treatments have been used to reduce neurotoxicity, including reduced-dose WBRT (rdWBRT) or high-dose chemotherapy with autologous stem cell transplant (HDC-ASCT). In this study, we characterized cognitive functions in PCNSL patients achieving long-term remission following rdWBRT or HDC-ASCT. METHODS: PCNSL patients completed cognitive evaluations at diagnosis, post-induction chemotherapy, and yearly up to 5 years following rdWBRT or HDC-ASCT. Quality of life (QoL), white matter (WM) disease, and cortical atrophy (CA) on MRI were assessed at similar intervals. RESULTS: Performance was impaired on most cognitive tests at diagnosis. Linear mixed model analyses in each group showed statistically significant improvement from baseline up to year 3 in attention/executive functions, graphomotor speed, and memory; however, there was a decline in attention/executive functions and memory after year 3 in both groups. WM abnormalities increased over time in both groups, but more patients treated with rdWBRT developed CA and WM changes. There were no significant longitudinal group differences in cognitive performance or QoL. CONCLUSIONS: Results indicated improvement in cognitive function up to 3 years post-treatment, but a decline at later time points and an increase in brain structure abnormalities in both groups. The findings suggest that rdWBRT and HDC-ASCT may be associated with delayed neurotoxicity in progression-free patients and underscore the need for long-term follow-up to characterize cognitive dysfunction in PCNSL patients.

Symptom relief during last week of life in neurological diseases.

OBJECTIVES: The aim of this study was to investigate symptom prevalence, symptom relief, and palliative care indicators during the last week of life, comparing them for patients with motor neuron disease (MND), central nervous system tumors (CNS tumor), and other neurological diseases (OND). MATERIAL & METHODS: Data were obtained from the Swedish Register for Palliative Care, which documents care during the last week of life. Logistic regression was used to compare patients with MND (n = 419), CNS tumor (n = 799), and OND (n = 1,407) as the cause of death. RESULTS: The most prevalent symptoms for all neurological disease groups were pain (52.7% to 72.2%) and rattles (58.1% to 65.6%). Compared to MND and OND, patients with CNS tumors were more likely to have totally relieved pain, shortness of breath, rattles, and anxiety. They were also more likely to have their pain assessed with a validated tool; to receive symptom treatment for anxiety, nausea, rattles, and pain; to have had family members receive end-of-life discussions; to have someone present at death; and to have had their family members offered bereavement support. Both patients with CNS tumor and MND were more likely than patients with OND to receive consultation with a pain unit and to have had end-of-life discussions. CONCLUSIONS: The study reveals high symptom burden and differences in palliative care between the groups during the last week of life. There is a need for person-centered care planning based on a palliative approach, focused on improving symptom assessments, relief, and end-of-life conversations.

Health-related quality of life of Brazilian children and adolescents with benign and malignant solid tumours: A prospective cohort study during the first year after hospital admission.

INTRODUCTION: This study aims to assess the impact of paediatric benign and malignant solid tumours and its treatment on the health-related quality of life of children and adolescents who were followed up in a Reference Center in Pediatric Oncology in Rio de Janeiro. METHODS: It is a prospective cohort study. Quality of life assessment was performed using the PedsQL™ 4.0 Generic Core Scales and PedsQL™ 3.0 Cancer Module protocols three times: during hospital admission (T1), 6 months after admission (T2) and 1 year after admission (T3). RESULTS: We evaluated 132 patients, 59 men and 73 women, aged 2-17 years. In PedsQL™4.0, the Emotional Functioning scale was the one with the worst scores, while the scores on the Social Functioning scale was the best. In PedsQL™ 3.0, the worst domains were Procedural Anxiety and Worry. Patients with malignant bone tumours had the worst health-related quality of life. The group who received only surgery had better results. Total scores of PedsQL™4.0 and PedsQL™ 3.0 improved between T1 and T3. CONCLUSION: Children and adolescents with malignant and benign neoplasms undergo changes in quality of life as a result of the disease and treatment, but an improvement has been observed over time.

Status and predictors of planning ability in adult long-term survivors of CNS tumors and other types of childhood cancer.

Long-term childhood cancer survivors' (CCS) quality of life can be impacted by late effects such as cognitive difficulties. Especially survivors of CNS tumors are assumed to be at risk, but reports of cognitive tests in CCS with survival times >25 years are scarce. We assessed planning ability, a capacity closely related to fluid intelligence, using the Tower of London. We compared 122 CNS tumor survivors, 829 survivors of other cancers (drawn from a register-based sample of adult long-term CCS), and 215 healthy controls (using sex-specific one-way ANOVAs and t-tests). Associations of CCS' planning ability with medical and psychosocial factors were investigated with a hierarchical linear regression analysis. Mean planning ability did not differ between CCS and controls. However, female CNS tumor survivors performed worse than female survivors of other cancers and female controls. CNS tumor survivors of both sexes had a lower socioeconomic status, and fewer of them had achieved high education than other survivors. In the regression analysis, lower status and anxiety symptoms were associated with poor planning, suggesting possible mediators of effects of disease and treatment. The results indicate the necessity to contextualize test results, and to include cognitive and psychological assessments into aftercare.

Development of the perceived barriers scale: a new instrument identifying barriers to career development and employment for young adult survivors of pediatric CNS tumors.

PURPOSE: Given the significant employment disparities for survivors of pediatric brain tumors, there is increased need to conduct vocational and career research with this group. The purpose of the present study was to construct an instrument, the Perceived Barriers Scale, that is psychometrically sound and has both clinical and research application related to career and employment issues of pediatric brain tumor survivors. METHOD: The participants consisted of 110 young adult central nervous system survivors of childhood cancer aged between 18 and 30 years old (M = 23.05, SD = 3.36) who were identified by the DFCI Pediatric Brain Tumor Clinic. The 12-item Perceived Barriers Scale was developed from a comprehensive literature review, clinical interviews conducted with survivors of pediatric brain tumors, and feedback from multidisciplinary providers. Exploratory factor analysis and correlations were completed to examine the initial psychometric properties of the scale. RESULTS: Exploratory factors analysis identified two factors that accounted for 57.92% with the two factors labeled as internal barriers and external barriers. All factors loaded significantly onto their respective factors (.48 to .88). The results of the correlational analysis found significant relationships between both internal barrier and external barrier subscales and CSE and WHODAS-2 providing initial support for the construct validity of the Perceived Barriers Scale. CONCLUSIONS: Overall, the study findings indicate good psychometrics with the brevity of the scale increasing potential application and utilization in both research and clinical settings. IMPLICATIONS FOR CANCER SURVIVORS: Identification of employment barriers for brain tumor survivors provides opportunity for more targeted vocational intervention.

Aspectos psicosociales de la calidad de vida en supervivientes pediátricos de tumores en el sistema nervioso central / Psychosocial aspects of the quality of life in pediatric survivors of tumors in the central nervous system

Introducción: Los supervivientes de tumores del sistema nervioso central (TSNC) presentan peor calidad de vida en relación a la salud (CVRS) que los supervivientes de otras neoplasias y la población normativa. El tipo y gravedad de las secuelas no parecen tener un impacto claro en la CVRS, pero la afectación del cociente intelectual (CI) sí podría modular la CVRS. Además, las estrategias de afrontamiento del paciente y de los progenitores y el malestar emocional parental podrían también actuar sobre la CVRS. Objetivo: Determinar si las estrategias de afrontamiento del adolescente y su CI, y las estrategias de afrontamiento, el estrés general y el malestar emocional parental, modulan las dimensiones psicosociales de la CVRS del superviviente. Método: Estudio transversal descriptivo-correlacional. Se reclutaron 37 adolescentes (12-19 años) que llevaban ≥1 año fuera de tratamiento oncológico y 44 progenitores. Resultados: En la CVRS valorada por los adolescentes, las dimensiones Relación con los Padres y Vida Familiar, Amigos y Apoyo Social, y Entorno Escolar dependen de la estrategia de afrontamiento de resolución del problema usada por el adolescente. La dimensión Aceptación Social depende del estilo de afrontamiento improductivo del adolescente y del estilo de búsqueda de ayuda social de los padres. La CVRS valorada por los padres depende de las secuelas, el CI del adolescente y, el tipo de tratamiento oncológico recibido. Discusión y conclusiones: Las estrategias de afrontamiento centradas en la resolución del problema, deben ser consideradas para valorar e intervenir en la mejora de la CVRS de los supervivientes TSNC

Introduction: Central Nervous System Tumor (CNST) survivors tend to show worse HRQoL than survivors of other types of cancer and general population. There are still many doubts about what variables and to what extend these are related to their HRQoL. Type and severity of late effects have not a clear impact on HRQoL. However, Intelligence Quotient (IQ) changes as a specific late effect of this sample population, could modulate their HRQL. Furthermore, coping from both patients and parents, as well as parental distress could play a significant role modulating HRQoL in these survivors. Aim: To study whether survivors' coping strategies and IQ, as well as coping, general stress and cancer-related distress of parents could modulate some psychosocial dimensions of their children HRQoL. Methodology: Correlational descriptive cross-sectional study. Thirty-seven teenagers (12-19 years old, both included) ≥1 year free of disease and 44 parents were recruited for the study. Results: When HRQoL is assessed by adolescents, the dimensions Parent Relations and Home Life, Social Support and Peer, and School Environment are related with the use of coping with problem style by adolescents. Social Acceptance/Bullying dimension is related with nonproductive coping by the adolescent and helpseeking coping style by parents. HRQoL assessed by parents is related with late effects, adolescents' IQ, and type of oncological treatment received. Discussion/Conclusion: Coping strategies, and specifically problem-solving strategies must be taken into account to assess and to intervene in the improvement of HRQoL of this sample population

Social adjustment in adolescent survivors of pediatric central nervous system tumors: A report from the Childhood Cancer Survivor Study.

BACKGROUND: The purpose of this study was to examine the prevalence and predictors of social difficulties in adolescent survivors of central nervous system (CNS) tumors. METHODS: Six hundred sixty-five survivors of CNS tumors (53.8% male and 51.7% treated with cranial radiation therapy [CRT]), who had a current median age of 15.0 years (range, 2.0-17.0 years) and were a median of 12.1 years (range, 8.0-17.7 years) from their diagnosis, were compared with 1376 survivors of solid tumors (50.4% male), who had a median age of 15.0 years (range, 12.0-17.0 years) and were a median of 13.2 years (range, 8.3-17.9 years) from their diagnosis, and 726 siblings (52.2% male), who had a median age of 15.0 years (range, 12.0-17.0 years). Social adjustment was measured with parent-proxy responses to the Behavior Problems Index. Latent profile analysis defined social classes. Multinomial logistic regression, adjusted for age, sex, and age at diagnosis, identified predictors of class membership. Path analyses tested mediating effects of physical limitations, sensory loss, and cognitive impairment on social outcomes. RESULTS: Caregivers reported that survivors of CNS tumors were more likely to have 0 friends (15.3%) and to interact with friends less than once per week (41.0%) in comparison with survivors of solid tumors (2.9% and 13.6%, respectively) and siblings (2.3% and 8.7%, respectively). Latent profile analysis identified 3 social classes for survivors of CNS tumors: well-adjusted (53.4%), social deficits (16.2%), and poor peer relationships (30.4%). However, 2 classes were identified for survivors of solid tumors and siblings: well-adjusted (86.2% and 91.1%, respectively) and social deficits (13.8% and 8.9%, respectively). CRT predicted class membership for CNS survivors (odds ratio [OR] for poor peer relationships, 1.16/10 Gy; 95% confidence interval [CI], 1.08-1.25; OR for social deficits 1.14/10 Gy; 95% CI, 1.04-1.25; reference, well-adjusted). Cognitive impairment mediated the association between all social outcomes and CRT (P values < .001). CONCLUSION: Almost 50% of survivors of CNS tumors experience social difficulties; the pattern is unique in comparison with solid tumor and sibling groups. Cognitive impairment is associated with increased risk, and this highlights the need for multitargeted interventions.

Public awareness of central nervous system tumors in the Kingdom of Saudi Arabia.

OBJECTIVE: To investigate individuals` knowledge about central nervous system tumors (CNST) signs and symptoms and risk factors, as well as their readiness to seek medical advice. The signs and symptoms associated with CNSTs are often vague, and failure to recognize them could lead to delays in seeking help and possibly fatal results. METHODS: This was a cross-sectional survey that utilized 2 delivery methods. A total of 1,500 personally delivered and 1,500 online self-administered questionnaires were completed in parallel between June 2015 and June 2016 for the occupants of the Kingdom of Saudi Arabia. RESULTS: Significant differences were observed for the sociodemographic characteristics of participants recruited via the 2 methods. The most recognized symptom was "Headaches" (45.2%), and the most recognized risk factor was "Radioactive location/occupation" (84.1%). Overall knowledge scores were low, significantly predicted by employment and cancer contact (p<0.05), while the scores significantly higher for participants who were willing to see their doctors within a week (p<0.005). The most recognized barrier to seeking help was "Worry about what the doctor might find" (74.0%). CONCLUSION: The level of awareness of CNSTs was low. Using a questionnaire delivered in 2 different ways enabled the recruitment of sample pools with different sociodemographic characteristics.

A prospective phase II trial of response adapted whole brain radiotherapy after high dose methotrexate based chemotherapy in patients with newly diagnosed primary central nervous system lymphoma-analysis of acute toxicity profile and early clinical outcome.

BACKGROUND: The treatment of primary CNS lymphoma (PCNSL) comprises high dose methotrexate (HDMTX) based chemotherapy followed by whole brain radiotherapy (WBRT), the major drawback of which is long term neurotoxicity. We intended to assess the feasibility of response adapted WBRT in PCNSL in the Indian setting. METHODS: We screened 32 patients and enrolled 22 eligible patients with PCNSL from 2015 to 2017 in a prospective phase II trial. The patients underwent five 2-weekly cycles of induction chemotherapy with rituximab, methotrexate, vincristine, procarbazine. Patients with complete response(CR) to induction chemotherapy were given reduced dose WBRT 23.4 Gy/13 fractions/2.5 weeks while those with partial response (PR), stable or progressive disease (SD or PD) were given standard dose WBRT 45 Gy/25 fractions/5 weeks. Thereafter two cycles of consolidation chemotherapy with cytarabine were given. The primary endpoints of the study were assessment of response rate (RR) and progression free survival (PFS). The secondary endpoints of the study were assessment of overall survival (OS), toxicity profile of treatment and serial changes in quality of life and neuropsychological parameters. RESULTS: Out of 19 patients who completed HDMTX based chemotherapy, 10 (52.63%) patients achieved CR, 8 (42.11%) patients had PR and 1 patient had PD. After a median follow-up period of 11.25 months, the estimated median OS was 19 months. The actuarial rates of PFS and OS were respectively 94.1 and 68.2% at 1 year and 50.2 and 48.5% at 2 years. Three patients in reduced dose WBRT arm had recurrence and two of them died of progressive disease, whereas there was no recurrence or disease related death in standard dose WBRT arm. On univariate analysis of PFS, age ≤ 50 years and use of standard dose WBRT (45 Gy) led to significantly improved outcome (p value 0.03 and 0.02 respectively). CONCLUSION: In patients with PCNSL, reduced dose WBRT after CR to HDMTX based chemotherapy may lead to suboptimal clinical outcome due to higher risk of recurrence, progression and early death. Trial Registration No CTRI/2015/10/006268.

Clinical routine assessment of palliative care symptoms and concerns and caregiver burden in glioblastoma patients: an explorative field study.

The implementation of self-reported outcome measurements into clinical routine was tested to help facilitate early access to palliative care (PC) for glioblastoma (GBM)-patients. Measures detail PC symptoms and concerns and caregiver burden. Between January 2014 and December 2016, a total of 337 GBM-patients were discussed during meetings of the neuro-oncology tumor board to examine further treatment options. Each patient, along with their caregivers, was requested to participate in self-assessment using the palliative outcome scale (POS) and the Zarit Burden Interview (ZBI). Analyses encompassed summary statistics, non-parametric tests, visual graphic analysis, content analysis and assessing the utilization of the specialized PC consulting service (SPCCS). Ninety-five (28%) GBM-patients and 71 (21%) caregivers completed the self-assessment. Of these, 20 patients and 12 caregivers repeated the assessment at least once more during follow-up. POS total scores were similar in the group of patients with initial diagnosis [10 (0-31)] and those with later disease stages like recurrent diagnosis [9 (0-25)], but ZBI total scores differed [14 (0-51) vs. 24 (2-62)]. Single item analysis demonstrated that anxiety and worries about the future predominated. Caregivers were torn between high engagement in caring and feeling overburdened. Still, requests for the SPCCS showed no increase. Actual implementation of measures like POS and ZBI for detecting PC concerns and caregiver burden with GBM-patients in the field remains challenging as indicated by the limited response rate and lack of increased requests for the SPCCS. Modified clinical routines including strengthening awareness of PC, and allowing proxy-assessment might help to overcome barriers.

Balancing relapses versus cognitive impairment in primary central nervous system lymphoma: a single-center experience.

OBJECTIVES: The outcomes of primary central nervous system lymphoma (PCNSL) are much improved with multi-modality regimens. Unfortunately, in limited-resource countries, chemo-radiotherapy is the only option of curative-intent treatment. This study aimed to evaluate the effects of low-dose whole brain radiotherapy (WBRT) as a consolidation on disease control and long-term neurocognitive functions. METHODS: We conducted a retrospective single-center study enrolling PCNSL patients from 2011 to May 2016 to evaluate the real-life treatment outcome and neurotoxicity from treatment especially radiotherapy. RESULTS: Thirty-seven newly diagnosed immunocompetent PCNSL patients were treated with a high-dose methotrexate-based regimen with or without WBRT. The median age was 56 (range 16-78) years old. After chemotherapy, the overall response and complete response (CR) rates were 59.5% and 43.2%, respectively. All 6 partial response (PR) patients and 6 of 16 CR patients underwent radiotherapy. In 22 patients who achieved CR, the progression-free survival (PFS) of patients without WBRT was significantly inferior to the WBRT group with the hazard ratio of 4.7 (95% confidence interval 1.14-19.82, p = 0.03). The 3-year PFS were 35% and 78.75%, respectively, but there was no difference in overall survival. The serial Montreal Cognitive Assessment evaluations (20-72 months post chemotherapy) of 10 long-term CR patients revealed one dementia among three patients without WBRT and five mild cognitive impairments in seven patients with WBRT. Except for the dementia case, all the other patients can perform daily activities without assistance. CONCLUSION: The low-dose WBRT consolidation is associated with lower PCNSL relapses with only mild neurocognitive toxicity.

Long-term survivors of primary central nervous system lymphoma.

Objective: In this study, we provide long-term outcome data of patients with primary central nervous system lymphoma. Methods: The long-term outcomes of PCNSL patients diagnosed between 1982 and 2006 were reviewed. Neurological late neurotoxicity symptoms, neuroradiological brain atrophy and leukoencephalopathy were evaluated. Surviving patients completed the Quality of Life Questionnaire-30 and Brain Cancer Module-20. The differences in overall survival were assessed using the Kaplan-Meier method and log-rank test. The differences between groups in terms of each investigated parameter were analyzed using the Wilcoxon signed-rank test. Results: Among 112 PCNSL patients, there were 33 (29.4%) long-term (> 5 years) survivors. The median survival of all long-term survivors was 105.7 months; of these, 8 (7.1%) were alive at the latest follow-up, with a mean survival time of 170.2 months (range, 121.8­286.4). Clinical assessment revealed severe neurotoxicity in 14 patients (42.4%), moderate neurotoxicity in 5 (15.1%), and normal status in 14 (42.4%). Correlations were seen between the neuroradiological imaging score changes and neurocognitive condition (P=0.0001), neurocognitive condition and the whole brain irradiation dose (P=0.0004), and atrophy and the whole brain irradiation dose (P=0.0035). Conclusions: A more severe clinical condition was found to be associated with increasing age and whole brain irradiation dose in long-term survivors with PCNSL.

Prevalence and associated positive psychological variables of anxiety and depression among patients with central nervous system tumors in China: a cross-sectional study.

BACKGROUND: Anxiety and depression have been identified as common psychological distresses faced by the majority of patients with cancer. However, no studies have investigated the relationship between positive psychological variables (hope, optimism and general self-efficacy) and anxiety and depression among patients with central nervous system (CNS) tumors in China. Our hypothesis is that the patients with higher levels of hope, optimism or general self-efficacy have lower levels of anxiety and depression when encountered by stressful life events such as CNS tumors. METHODS: Questionnaires, including the Hospital Anxiety and Depression Scale, the Herth Hope Index, the Life Orientation Scale-Revised and the General Self-Efficacy Scale, and demographic and clinical records were used to collect information about patients with CNS tumors in Liaoning Province, China. The study included 222 patients (effective response rate: 66.1%). Hierarchical linear regression analyses were performed to explore the associations among hope, optimism, general self-efficacy and anxiety/depression. RESULTS: Prevalence of anxiety and depression were 42.8 and 32.4%, respectively, among patients with CNS tumors. Hope and optimism both were negatively associated with anxiety and together accounted for 21.4% of variance in anxiety. Similarly, hope and optimism both were negatively associated with depression and accounted for 32.4% of variance in depression. CONCLUSIONS: The high prevalence of anxiety and depression among patients with CNS tumors should receive more attention in Chinese medical settings. To help reduce anxiety and depression, health care professionals should develop interventions to promote hope and optimism based on patients' specific needs.

Clinical characteristics and late effects in CNS tumours of childhood: Do not forget long term follow-up of the low grade tumours.

AIM: To investigate clinical characteristics and late effects of CNS tumours in childhood with a special focus on low-grade tumours, especially low-grade astrocytoma and glioneuronal tumours. METHODS: A retrospective population based study was performed at Uppsala University Children's Hospital, a tertiary referral centre for children with CNS tumours. Patients were identified from the National Brain Tumour Registry and the National Epilepsy Surgery Registry. Hospital medical records were analysed for patients with a follow up of ≥5 years after diagnosis. A re-evaluation of the neuro-pathological diagnosis was performed. RESULTS: A total of 193 patients (age 0-17.99 years) during a twelve-year period (1995-2006) were included; 149 survived ≥5 years. Three larger subgroups could be identified: astrocytic, embryonal and glioneuronal tumours. A supratentorial location was found in 52%. Medical late effects were mainly neurological and endocrinological, affecting 81% and 26% of surviving patients. Cognitive late effects were a frequent finding in the whole group but also in low-grade astrocytoma and glioneuronal tumours (53% and 67%). Thirty per cent had some kind of pedagogic support in school. CONCLUSION: Late effects are common in long-term survivors of CNS tumours in childhood. Low-grade astrocytoma and glioneuronal tumours are no exception, and the findings support the need for long-term follow up.