Abstract Background Atypical teratoid rhabdoid tumours (ATRT) are aggressive brain tumours mostly occurring in early childhood. Largest published series arise from registries and institutional ...experiences (1–4). The aim of this report is to provide population-based data to further characterise this rare entity and to delineate prognostic factors. Patients and methods A national retrospective study of children ⩽18 years diagnosed with a central nervous system (CNS) ATRT between 1995 and 2007 was undertaken. All cases underwent central pathology review. Results There were 50 patients (31 males; median age at diagnosis of 16.7 months). Twelve patients were >36 months. Infratentorial location accounted for 52% of all cases. Nineteen patients (38%) had metastatic disease. Fifteen (30%) underwent gross total resection (GTR). Ten patients (20%) underwent palliation. Among the 40 remaining patients, 22 received conventional chemotherapy and 18 received high dose chemotherapy regimens (HDC); nine received intrathecal chemotherapy and 15 received adjuvant radiation. Thirty of the 40 treated patients relapsed/progressed at a median time of 5.5 months (0–32). The median survival time of the entire cohort was 13.5 months (1–117.5 months). Age, tumour location and metastatic status were not prognostic. Patients with GTR had a better survival (2 years overall survival (OS): 60% ± 12.6 versus 21.7% ± 8.5, p = 0.03). HDC conferred better outcome (2 years OS 47.9% ± 12.1 versus 27.3% ± 9.5, p = 0.036). Upfront radiation did not provide survival benefit. Six of the 12 survivors (50%) did not receive radiation. Conclusion The outcome of CNS ATRT remains poor. However, the use of HDC provides encouraging results. GTR is a significant prognostic factor. The role of adjuvant radiation remains unclear.
Background
Recent changes to the legal status of marijuana in Canada warrant a review of the information that patients and families are accessing online regarding the role of cannabis in cancer. The ...aims of the current research were to identify the quality of literature available online as well as the themes, and opinion (i.e., pro-, neutral, or anti-cannabis) of online articles.
Methods
Searches were conducted using three primary search engines: Google, Yahoo, and DuckDuckGo. Articles were assessed for quality based on a modified scale for evaluating online sources. Content of all unique articles was coded using a qualitative thematic methodology in a line-by-line fashion. Codes were clustered to determine themes within articles. Finally, opinions were determined by examining all articles in a line-by-line fashion. Each statement was coded as either pro-cannabis (positive) or anti-cannabis (negative).
Results
We found most articles were authored by journalists (39.4%) and MDs (14.1%) and published as news (35.2%) or web articles (28.2%). The content of articles focused on four themes: the reasons for and against cannabis use; the opinions of health care providers; the restrictions placed by governing bodies and the need for additional research, education, and standardization. Article opinions were neutral-pro-cannabis.
Conclusions
Health care providers should be aware that the overall quality of information found online is considered “satisfactory.” The majority of articles present a pro-cannabis opinion.
Atypical teratoid rhabdoid tumour (ATRT) is a malignant tumour of the central nervous system with a dismal prognosis. There is no consensus on optimal treatment and different multimodal strategies ...are currently being used in an attempt to improve outcomes. To evaluate the impact of high-dose chemotherapy followed by autologous stem-cell rescue (HD48 SCR), radiotherapy (RT) at first line, intrathecal chemotherapy (IT) and extent of surgical resection upon recurrence-free survival (RFS) and overall survival (OS). An online database search identified prospective and retrospective studies focused on the treatment of children and adolescents with newly diagnosed ATRT. Clinical, therapeutic and outcome data were extracted and an individual pooled data analysis was conducted. Out of 389 publications, 12 manuscripts were included in our review. Data from 332 patients were analysed. Median age at diagnosis was 37 months (range 1–231). HD-SCR, RT and IT had been administered to 28.6 % (58/203), 49.6 % (118/238) and 21 % (65/310) of the patients, respectively. Gross total resection (GTR) had been achieved in 46.5 % (152/327) of the cases. In the multivariate analysis, hazard ratios (95 % Confidence Interval) for HD-SCR were: RFS-HR = 0.570 (0.357–0.910) p = 0.019, and OS-HR = 0.388 (0.214–0.704) p = 0.002; and for RT: RFS-HR = 0.551 (0.351–0.866) p = 0.01, and OS-HR = 0.393 (0.216–0.712) p = 0.002. IT and GTR were not significantly associated with improved RFS or OS in the multivariate analysis. In our pooled data review, HD-SCR and RT at first line were associated with improved outcomes in children and adolescents with newly diagnosed ATRT.
The treatment of children with cancer is associated with significant burden for the entire family. Frequent clinic visits and extended hospital stays can negatively affect quality of life for ...children and their families.
Here, we describe the development of a Hospital at Home program (H@H) that delivers therapy to pediatric hematology, oncology, and blood and marrow transplant (bmt) patients in their homes. The services provided include short infusions of chemotherapy, supportive-care interventions, antibiotics, post-chemotherapy hydration, and teaching.
From 2013 to 2015, the H@H program served 136 patients, making 1701 home visits, for patients mainly between the ages of 1 and 4 years. Referrals came from oncology in 82% of cases, from hematology in 11%, and from bmt in 7%. Since inception of the program, no adverse events have been reported. Family surveys suggested less disruption in daily routines and appreciation of specialized care by hematology and oncology nurses. Staff surveys highlighted a perceived benefit of H@H in contributing to early discharge of patients by supporting out-of-hospital monitoring and teaching.
The development of a H@H program dedicated to the pediatric hematology, oncology, or bmt patient appears feasible. Our pilot program offers a potential contribution to improvement in patient quality of life and in cost-benefit for parents and the health care system.
Concerns about radiotherapy-related neurocognitive sequelae in young children have led to deferral or avoidance of radiation in contemporary treatment for this fragile group of patients. We compared ...survival and neurocognitive outcome in two groups of infants with medulloblastoma who received adjuvant conventional craniospinal irradiation (CSI) or reduced or no radiotherapy during an era of change in the philosophy of infant medulloblastoma treatment.
From 1985 to 2007, 29 patients 3 years of age or younger were diagnosed and treated with curative intent in our institution. Children treated before 1994 received adjuvant radiation with chemotherapy; subsequently, radiation was prescribed essentially for disease progression or relapse.
Median age at diagnosis was 24 months (range: 1-36 months); 15 patients (52%) presented with metastatic disease at diagnosis. As part of initial treatment, 8 children received adjuvant radiotherapy with chemotherapy, and 21 children received postoperative chemotherapy only. Five children treated with chemotherapy alone are in prolonged remission. The 5-year event-free and overall survivals were 35.9% +/- 9.8% and 50.2% +/- 9.6% respectively. Extent of resection, metastatic status, and desmoplastic histology were not found to be significant prognostic factors. On serial neurocognitive evaluations, patients treated with chemotherapy with or without reduced radiotherapy demonstrated improvement of intellectual function over time. Patients treated with conventional csi exhibited significantly lower intelligence quotient scores and academic performance, with the exception of receptive vocabulary.
Avoidance of conventional CSI in treatment of very young children with medulloblastoma appears to be associated with a preserved neurocognitive profile. Neurocognitive evaluation should be integrated into the primary objectives of future infant protocols.
Embryonal tumors are a heterogeneous group of central nervous system (CNS) tumors whose subgroups have varying incidence and outcome. Despite these differences, they are often grouped as a single ...entity for study purposes. To date, there are no Canadian multi-institutional studies examining the incidence and outcome of all embryonal subtypes. The current study is an observational study reviewing embryonal tumors in all patients less than 36 months of age diagnosed with a CNS tumor in Canada from 1990 to 2005. Embryonal tumors accounted for 26.9% of all CNS tumors. Medulloblastomas were the highest proportion of the embryonal tumors at 61.5%. Atypical teratoid/rhabdoid tumors (AT/RT) had the second highest proportion of embryonal tumors at 18%. The proportion of primitive neuroectodermal tumors (PNET) was 16%, with 2.6 and 1.9% for congenital medulloepithelioma and ependymoblastoma tumors, respectively. AT/RT and PNET were more common in younger age groups. Medulloblastoma became more prevalent with increasing age, with its highest prevalence in the 25 to 36 month age group. Survival rates for our Canadian population at 18 and 24 months were 0.74 and 0.68 for medulloblastoma, 0.64 and 0.60 for PNET, and 0.36 and 0.29 for AT/RT, respectively. Overall, our data are comparable with published international rates for embryonal tumors. These incidence and outcome figures can guide future research into these rare tumors.
BACKGROUND: High dose chemotherapy strategies were developed to avoid craniospinal irradiation and prevent unacceptable neurotoxicity in young children. However, long-term outcome, including ...neurocognitive outcome, of this approach has not been widely described. METHODS: This retrospective study collected data from 7 institutions, on young children with medulloblastoma who received high-dose Carboplatin, Thiotepa according to the protocol CCG99703 between 1998-2012. Data on pathology, molecular subgrouping, chemotherapeutic, radiation, ototoxicity, neurognitive evaluations and survival were collected. RESULTS: There were 53(30 males) patients diagnosed at a median age of 24 months(2.9-63.2). Twenty(37.7%) had metastatic disease and 35(66%) underwent gross total resection(GTR). Seventeen(32.1%) had nodular desmoplastic(ND) subtype. Twenty two(56.4%) out the 39 patients with molecular subtyping belong to the SHH group. Three patients received intrathecal chemotherapy, 6 received HD MTX during induction and 7 underwent maintenance chemotherapy. Seventeen patients received radiation, including only 9(16.9%) in an adjuvant setting. Complete continuous remission(CCR) after induction and consolidation were respectively 73.6% and 77.4 %. Two patients died of treatment related toxicity. Forty-two patients are alive at a median follow-up of 3.7 years from diagnosis with a projected 5-year PFS and OS of respectively 69.2%( plus or minus 7) and 76.1%( plus or minus 6.5). ND histology, SHH subgroup and CCR were significantly associated better PFS, but only CCR post induction remained significant for better OS. Non irradiated children had a better PFS compare to those who received radiation(5y PFS 84.6% versus 42.2%p = 0.007). Severe ototoxicity( greater than or equal to Brock grade 3) was present in 13.2% of 31 evaluable patients. Thirteen required hearing support. Neurocognitive assessements were available in 24 survivors(57%). Mean FSIQ for the cohort was 91(range 67-119). CONCLUSION: Young children with MB treated with this strategy have a very encouraging OS (76.4 %) while only 17% of the patients who received adjuvant radiation. Although the ototoxicity was significant, neurocognitive profile of the survivors appears to be within normal range.
PDF
