Survival of children with medulloblastoma in Canada diagnosed between 1990 and 2009 inclusive

Abstract

The treatment of medulloblastoma, the most common malignant brain tumor in children, has evolved over the last few decades. The objectives of this paper were to determine the survival of pediatric medulloblastoma in Canada, to determine if there has been an improvement in the survival rates between the years of 1990 and 2009, inclusive, and to determine prognostic factors for survival. All patients under the age of 18 years diagnosed with medulloblastoma from 1990 to 2009, inclusive, in Canada were included. Data collected included date of diagnosis, age at diagnosis, gender, stage, pathology, treatment, recurrence and current status. From these, survival rates were determined. Data were obtained on 628 eligible patients. The overall 5-year survival rate for the study time period was 69.2 ± 3.3 %. The survival rate increased during the interval of 1996-2000, then remained stable; 1990-1994: 60.2 ± 4.3 %; 1995-1999: 73.2 ± 3.5 %; 2000-2004: 68.8 ± 3.7 %; and 2005-2009: 72.1 ± 4.9 %, p = 0.05. Children over 14 years of age had a significantly better overall survival than those age 5-14 and those under 5 (85.7 ± 5.5 % vs 76.1 ± 2.7 % and 60.8 ± 3 % respectively, p = 0.001). Histologic medulloblastoma subtype and M stage of disease did not result in significant differences in survival. Despite changes in approaches to therapy, we demonstrate a steady survival rate for children with medulloblastoma after 1996. In our analyses, age over 14 years was associated with a higher survival rate.

Children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor in Norway from 1974 through 2013: Unexplainable regional differences in survival

“…Survival rates in the herein presented consecutive national Norwegian MB/CNS‐PNET material were in line with a similar study from Sweden, but lower than results from other countries (Canada and France) . One possible explanation is that the Canadian work did not include patients from the period 1974–1990.…”

Medulloblastoma in the age of molecular subgroups: a review

“…41 Patients not meeting the aforementioned criteria were classified as high risk, with survival rates ranging from 60% to 65%. 12,20 Recently, an updated risk stratification proposal for medulloblastoma patients ages 3-17 was developed (Fig. 3), taking into account subgroup status and select genetic and cytogenetic aberrations to more accurately predict outcome.…”

“…Trade-offs between the side effects of current therapies and the potential improved survival have caused survival rates to be relatively stagnant over the past three decades. 20 Resection can be complicated by neurological deficits, particularly posterior fossa syndrome (cerebellar mutism), which occurs in approximately 25% of patients and characteristically manifests as difficulty with language production and emotional lability. 28 Radiotherapy and cytotoxic chemotherapy, particularly in younger children, are associated with neurocognitive impairment, hearing loss (potentially exacerbated by cisplatin chemotherapy), short stature, pituitary hormone deficiency, cataracts, cerebrovascular disease (e.g., stroke, intracranial hemorrhage, cavernous malformations), and secondary malignancies.…”

“…Despite such aggressive multimodal therapy, approximately 30% of patients eventually succumb to this disease, and survivors cope with the long-term side effects of treatment that have significant impacts on their quality of life. 12,18,20,35,41,42,66 Initial descriptions of medulloblastoma date back to Harvey Cushing, who initially described medulloblastoma as a subset of gliomas ( Fig. 1).…”

“…16 Finally, the addition of cytotoxic chemotherapy regimens as standard of care, starting with trials in the 1970s, improved the 5-year survival risk for average-risk disease to 70%-80% and for high-risk disease to 60%-65%. 5,20,41 Rapid advances in molecular genetics over the past two decades have provided significant advancements in our understanding of medulloblastoma. This tumor entered the molecular era, beginning with gene expression array studies demonstrating that medulloblastoma is a distinct entity from other embryonal central nervous system tumors.…”