by A team of researchers from The Institute of Cancer Research, London, in collaboration with a European working group, have made significant strides in understanding the unique characteristics of gliomatosis cerebri, a rare and aggressive form of childhood brain tumor. Their findings, published in the journal Neuro-Oncology, could lead to more effective treatment options for this elusive condition.
Gliomatosis cerebri is a type of glioma, a Brain Tumor that originates from the glial cells, which support the brain and spinal cord. Unlike most gliomas, gliomatosis cerebri does not form a solid tumor but instead presents as a complex network of cancerous threads that infiltrate deep into the brain. This spider web-like structure makes surgical removal challenging and radiotherapy difficult to administer, while current chemotherapy treatments have proven ineffective.
Approximately 100 cases are diagnosed in the United States each year, with most patients surviving less than a year. Despite its initial identification in 1938, the definition, underlying biology, and treatment of gliomatosis cerebri remain unclear. Previous research has failed to identify a distinct molecular signature that sets it apart from other gliomas in both children and adults.
The researchers’ decade-long study aimed to comprehensively analyze this rare condition, providing new insights into its complex nature. Gliomatosis cerebri is currently described by the World Health Organization as a special growth pattern of various types of diffuse gliomas, rather than a distinct disease. This lack of understanding has hindered the development of targeted therapies.
The team’s findings could potentially change the way gliomatosis cerebri is classified and treated, paving the way for more effective interventions and ultimately improving outcomes for affected children.
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1. Source: Coherent Market Insights, Public sources, Desk research
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