In this E-Learning module, the author provides basics in terms of epidemiology, histologic subtypes, molecular biology, diagnosis, staging and multidisciplinary treatment of medulloblastoma and illustrates key differences in terms of age onset of the disease.
In adults, the incidence of medulloblastoma is 0.6 to 1 patient per million per year, representing 1% of central nervous system tumours, with a median age at onset between 25 and 30 years. In principle, disease-free survival rate at 10 years is excellent; however, long term sequelae from treatment are present.
The author presents the evidence from studies in paediatric and adult patients and highlights key differences related to age. The clinical practice guidelines for diagnosis, treatment and follow-up of post-pubertal and adult patients with medulloblastoma are also highlighted and differences in outcomes and the applicability of the paediatric experience put into context.
The author underlines that medulloblastoma is a radiosensitive and chemosensitive disease. A correct staging is the mainstay of its treatment. The treatment strategies presented in this module are supported by well-designed algorithms. Clinical prognostic factors, differences related to histology, molecular biology and outcomes depending on age, are featured, as well as expected survival based on molecular subtypes.
The author also elaborates on the evidence from children and adolescents in terms of chemotherapy toxicity in adult medulloblastoma. In adult patients, there is no issue of growth impairment, but chemotherapy after craniospinal irradiation in adult patients leads to long term side effects. The toxicity of paediatric protocols in adult patients is also highlighted.
Finally, the module considers in detail molecular targeted therapies, particularly SHH inhibitors, and illustrates the development of randomised clinical trials for adult patients with medulloblastoma.
