Gliomatosis Cerebri: A Ghost in the Brain
Gliomatosis Cerebri: A Ghost in the Brain
Medscape: How do you treat pediatric GC patients in the meantime?
Dr. Phillips: We have enough patients in whom radiation therapy appears to have slowed the progression to convince us that radiation therapy plays a role. The advantages of chemotherapy have been anecdotal, and we cannot say with confidence that the addition of temozolomide, combinations of bevacizumab and irinotecan, or any other drug active in malignant glioma provides a benefit in pediatric GC. We have acted as if this is a malignant glial neoplasm and treated it with whatever has been the best available therapy for adult malignant gliomas, but to be intellectually honest, we have not found any chemotherapy that has been effective in GC.
Medscape: How likely is it that whole-genome sequencing will be done?
Dr. Phillips: I think the prospects are good. It takes a lot of money to do the sequencing right now, but that will get cheaper. This is an uncommon entity; therefore, no single institution is likely to have enough patients, so it will require a major cooperative effort. It will also require extremely careful specimen collection, strictly referred by pathologist, with blood specimens you could get lymphocytes from, and with certification from a competent and experienced neuropathologist that what you are looking at is actually GC.
We will also need clinical data, preferably longitudinal, that includes survival data, response data, and radiographic characteristics. And, ultimately, neuropathology is going to become the appropriate use of molecular tools that improve interobserver reliability. Right now what we're depending on is, "Well, it looks like this to me..."
What effective treatments do we have in malignant gliomas? Not many, and the ones we have are not curative. I don't think we will make much progress in GC until we have defined the genomic changes and begin to understand the critical pathways that lead to uncontrolled growth in these tumors.
Whole-Genome Sequencing: Key to Effective Therapy?
Medscape: How do you treat pediatric GC patients in the meantime?
Dr. Phillips: We have enough patients in whom radiation therapy appears to have slowed the progression to convince us that radiation therapy plays a role. The advantages of chemotherapy have been anecdotal, and we cannot say with confidence that the addition of temozolomide, combinations of bevacizumab and irinotecan, or any other drug active in malignant glioma provides a benefit in pediatric GC. We have acted as if this is a malignant glial neoplasm and treated it with whatever has been the best available therapy for adult malignant gliomas, but to be intellectually honest, we have not found any chemotherapy that has been effective in GC.
Medscape: How likely is it that whole-genome sequencing will be done?
Dr. Phillips: I think the prospects are good. It takes a lot of money to do the sequencing right now, but that will get cheaper. This is an uncommon entity; therefore, no single institution is likely to have enough patients, so it will require a major cooperative effort. It will also require extremely careful specimen collection, strictly referred by pathologist, with blood specimens you could get lymphocytes from, and with certification from a competent and experienced neuropathologist that what you are looking at is actually GC.
We will also need clinical data, preferably longitudinal, that includes survival data, response data, and radiographic characteristics. And, ultimately, neuropathology is going to become the appropriate use of molecular tools that improve interobserver reliability. Right now what we're depending on is, "Well, it looks like this to me..."
What effective treatments do we have in malignant gliomas? Not many, and the ones we have are not curative. I don't think we will make much progress in GC until we have defined the genomic changes and begin to understand the critical pathways that lead to uncontrolled growth in these tumors.
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