Neuroblastoma: Update Course 2014
With Dr. CCHMC Pediatric Surgery · hosted by Dr. Dan von Almen · StayCurrentMD
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Survival rate for high-risk neuroblastoma patients is in the 38 to 40% range
Surgeon-reported degree of resection showed only 66% concordance with radiologist assessment of postoperative imaging in tandem transplant pilot study
Repeat study in recent COG high-risk trial showed same 66% concordance rate between surgeon op notes and postoperative imaging, with surgeons underestimating and radiologists overcalling resection completeness
More chemotherapy or other agents like MIBG may make tumor more fibrotic and make subadventitial dissection more difficult
Complication rate for aggressive neuroblastoma resection is approximately 30% morbidity with mortality less than 1%
Riley Hospital study showed only survivors with stage 4 neuroblastoma were those who had primary tumor resection
High-risk neuroblastoma patients receive 9 different drugs, bone marrow transplantation, and some receive total body radiation
High-risk neuroblastoma treatment includes tandem peripheral blood stem cell transplants, with second transplant given as soon as patient recovers from first, followed by immunotherapy and Retin-A
High-risk neuroblastoma survival increased from 10% to 30-40% with treatment intensification
COG 3973 study's inability to demonstrate overall survival benefit may be type 2 error due to smaller sample size (230 vs 1,300 patients in European study)
Aggressive neuroblastoma resections using subadventitial dissection should not be performed by occasional pediatric oncological surgeons doing one case per year or every other year
Immunotherapy is effective in neuroblastoma in the setting of minimal residual disease
Memorial Sloan Kettering data suggests biggest volume response of neuroblastoma tumor occurs with first two cycles of chemotherapy, with very little response after that
Sloan Kettering study suggests stage 4 neuroblastoma patients do better with primary tumor resection
Kylie's study showed no difference in survival based on completeness of neuroblastoma resection
European studies show no difference in survival based on completeness of neuroblastoma resection
Most recent COG study shows probably no difference in survival based on completeness of neuroblastoma resection
Georgia study showed 46% survival in high-risk neuroblastoma with transplantation plus immunotherapy plus differentiating agents
Many deaths in intensified high-risk neuroblastoma treatment were due to the treatment itself rather than cancer
German study of 278 stage 4 high-risk neuroblastoma patients achieved complete resection in almost half and >90% resection in another quarter (75% total with >90% resection)
German study showed overall survival 45%, event-free survival 33%, and local progression-free survival 58% in stage 4 neuroblastoma
German study showed no difference in overall survival, event-free survival, or local progression-free survival based on completeness of resection in stage 4 neuroblastoma
German study concluded aggressive surgery is not justified in stage 4 neuroblastoma, that limited operations decrease complications, and there is limited impact on patient outcome
European study included 1,324 high-risk neuroblastoma patients (stages 2, 3, and 4) and achieved 76% with >95% resection
European neuroblastoma study showed 0.5% mortality and 10% morbidity (30% including lesser complications)
European study showed significant improvement in event-free survival and overall survival with aggressive resection in high-risk neuroblastoma - first study to show overall survival benefit
European study concluded >95% resection results in improvement in event-free survival in high-risk neuroblastoma
COG 3973 study of approximately 230 high-risk neuroblastoma patients showed significant improvement in local relapse-free survival and event-free survival but not overall survival
European neuroblastoma procedures are performed in more than 200 hospitals yet still demonstrated survival improvements
Older data shows worse survival in neuroblastoma when kidney is removed, likely because single kidney limits chemotherapy dosing