false
Catalog
2018 AANS Annual Scientific Meeting
Medulloblastomas in the Era of Molecular Biology: ...
Medulloblastomas in the Era of Molecular Biology: A Challenge for Developing Countries
Back to course
[Please upgrade your browser to play this video content]
Video Transcription
So I first invite Ibaki to the stage to talk about medulloblastomas and the area of molecular biology and the challenge for developing countries. I look forward to your talk. Thank you for joining us. Ibaki is from Algeria. So dear all, thank you to the organizing committee of the AANS to invite us to share with you the experience of African neurosurgeons in pediatric neurosurgery. So today I want to share with you some reflections that I have as a neurosurgeon coming from a developing country about a very well-known disease, which is the medulloblastoma. I have no disclosure. This is my country. It's the biggest country in Africa. And we can see that we have a free healthcare system. So everybody knows what is a medulloblastoma. We know that we can achieve 70% of overall survival at five years in all the categories of risk. But we know also that the response is heterogeneous. And of course, that we have a great morbidity due to the treatment that we are giving to these patients. So the question is, can we limit the morbidities while we can give a treatment to have a better response to the treatment? So this is the current standard treatment. Surgery, of course, maximum safe resection. And also radiotherapy, chemotherapy. And the most important that we give this treatment according to a risk stratification. So in Algeria, since 2013, we have adopted the risk stratification involving histology. And so all the medulloblastoma of large cells and anaplastic are considered as high risk, even if they are in R0 and M0. But what we know now is that since 2006, since the first paper of Taylor, that we have had an explosion of knowledge in the field of molecular biology. And all these papers and many other papers were showing us why we have a heterogeneous response to treatment. And finally, in 2012, we have had this consensus concerning the four biologic groups with the Wnt, Sonic Hedgehog, Group 3, and Group 4. And we understand that they are different concerning demographics, concerning clinical features, histology, and genetic signature. But furthermore, they are very different concerning prognosis, since we know that the prognosis in Wnt is excellent, while it is very dismal in the Group 3. So that's why this data, that they were very important, and they have been integrated in the WHO tumor classification in 2016. And this is the new classification. And you can see that we have a new category, which is called medulloblastoma otherwise specified. So we will talk about it after that. And all this data are very important, because we can now better select our patient concerning treatment and, of course, maybe predict the prognosis. So these are the implications of advances in molecular biology. We will have certainly new risk certification. We will have risk certification with clinical data, but, of course, with molecular informations. And we will have probably new categories of risk. So targeted therapy, of course, because we know now the signaling pathway. And all that will give us new protocols with sometimes de-escalade and sometimes intensification. We know that we have medulloblastoma with good prognosis that are probably over-treated. And maybe these patients could be spared by reducing their treatment. On another hand, we have medulloblastoma with very, very poor prognosis. And they will die in whatever you will do. So maybe we can make a reduction of therapies to improve the quality of life without modification of prognosis. So let's talk about surgery. Will we operate all medulloblastoma samely? Probably not. And maybe we will operate some patients, very young babies with huge tumor like this, with metastasis, with partial resection in the goal to preserve the quality of life. And regarding the first study comparing the extension of resection among the four group, it appears that the extension of resection is, the difference is statistically significant between gross total resection and subtotal resection only in the group four. So maybe we have to make a reflection about that. Till now, maximal resection is still a standard. But, of course, without increasing morbidity. And don't forget that a gross total resection of 100% and a subtotal resection of 90% doesn't have a difference concerning prognosis. Reducing doses of irradiation, probably yes. With, in the Wnt group, with this study that is ongoing with the International Society of Pediatric Oncology. And they are trying to give to the Wnt, R0, M0, only 80 gray of irradiation. Instead, 23 gray. And I think that we will have good results with this study. Chemotherapy alone, of course, it's advocated in young babies with sonic hedgehog medulloblastoma. Of course, with ejection of entratical chemotherapy. And maybe these babies will be spared, of course, by of irradiation. Targeted therapy, yes. When we look at this slide, there is a rationale to give a target therapy in medulloblastoma. But when we look at this multiple genes, we can understand that sometimes we can have downstream resistance and cross between two signaling pathways. So, these are the different trials that have been done or in preclinical or achieved. But we have to know that the most study is the sonic hedgehog pathway. And now we are at the second generation of inhibitors of sonic hedgehog. And maybe we can solve the resistance downstream, the small gene. But things are getting more complicating in 2017. Because now we know that these four subgroups have also all subgroups, and we have now 12 subgroups. So, let me talk about what is happening in developing countries. Just to remember that we can make the diagnosis of the biologic subgroups by immunohistochemical study. But we can just make the difference between Wnt, sonic hedgehog. But the group three and group four are in a group which is called non-Wnt, non-sonic hedgehog. So, until now, we don't have immunohistochemical markers to do the diagnosis between the two entities. So, what we need for immunohistochemical markers, that beta-catenin, GAB1, YAP1, P53, and P77 and GFR. What we have in Algeria is beta-catenin, GAB1, YAP1, and P53. So, we have many immunohistochemical markers that are available in our country. But unfortunately, we can't make the difference between the group three and group four because we don't have fish available for medulloblastoma. It's available for lung cancers, for other cancers of adult, but not for brain tumors of children. So, the situation in Africa, for Morocco, the situation is quite the same as in Algeria. Tunisia, they have only beta-catenin and fish is available, but not for medulloblastoma. Egypt, they have only one center that where everything is available. And my colleagues from South Africa are doing with conventional histology, but they are trying to set up a national plan for molecular subgroupings, but they are looking also for funds. For the sub-Saharan Africa, many, many countries don't have immunohistochemistry. So, the consequences that we, as neurosurgeons from developing countries, we will not talk the same language as you in developed country. We will have more and more medulloblastoma or otherwise specified. We can't benefit from the advances in treatment and our patient will be exposed to complications that can be avoid. So, what can we can do? Developed countries have a duty to assist the developing, the poor income countries. And for the medium-income countries, we have to convince the politicians and the investors to develop this molecular biology, particularly in the field of brain tumors in children. In Algeria, we can achieve something with the Pediatric Oncology Group of Algiers. We can add the brain tumors of children to the cancer plan in Algeria. And I hope that we'll give more tools to solve our problems. And we can also pry. So, just to meditate, look at what we have spent of money in armament around the world and what it can represent in the field of health. So, things are going quickly. It is time to adapt. And this beautiful sentence of Winston Churchill is never, never, never give up. I think that we will do that. We must do that in the developing countries. And thank you. This is the Pediatric, the Neuro-Oncology Group of Algiers. You can see that is a female group. And I just want to show you these flyers about Congress, which are very important for us. The Mediterranean Association of Neurological Surgeons Congress, which will be held in Naples. The Cairns Congress, which will be held in Abuja, in July. And finally, the Pan-Arab Neurosurgery Meeting in Marrakesh in October. Thank you very much. Thank you very much for a very interesting talk and very comprehensive. And also, thank you for staying on time perfectly. We have time for a few questions. I would just ask that Dr. Mahmood, please come to the front of the room just so we can get set up. But please, questions for Dr. Bhakti. And if you have questions, please use the microphone. I can start, Mark. You know, beautiful talk. So maybe just give us a flavor for, you know, how many pediatric neurosurgeons are in Algeria? Is there one center? Or if there are tumors done in other areas, is there some central review that, how do you get this kind of funding or convince some funding agency to have some collective or some initiative that you're doing? Or is it all done at one center? Can you maybe give us flavor for that? In Algeria, we don't have pediatric neurosurgery. We are neurosurgeons. And we have, of course, in all the big centers, unit divisions of pediatric neurosurgery. I am general neurosurgeon, but these 10 years I'm doing only pediatric neurosurgery. And by the group of pediatric neuro-oncology group, we have all the information of brain tumors in children in Algeria. So we can work together and give better, I don't know how we can say it, better management of brain tumors in children. And this group exists since six years. Great. No, it's terrific. And my feeling is sometimes it's challenging, at least from my time in Uganda, it's very different. But, you know, it's very, you get these kids in quite late stage of their disease where they haven't been able to get imaged. It's been very hard to get an MRI scan. Is that an issue? No, no. We don't have sedated scans. No, the problem is not in this field. We don't have problems for imaging also to operate patients. The problem is now in Algeria, of course, and Morocco is the same. And North Africa is quite the same, and I think also South Africa. The problem is not in imaging. It's not in operate patient. It's after, for the adjuvant therapy. Great. Thank you very much. One more question, please. This is about radiotherapy. Is there any radiotherapy that can be applied to these kids? Is there any section that you have to do? What do you have to do? How can you give people radiation in spite of overage? At my knowledge, the standard in radiotherapy for medulloblastoma is still conformational radiotherapy. So, of course, we don't have radiosurgery until now in Algeria, but it's not necessary for this medulloblastoma since the standard is conformational radiotherapy. Right, and that would be our standard. Also, the question was about using, I believe, radiosurgery for medulloblastoma, and in our practice, like in yours, we would use conformal fields for that as a standard. Any other questions? And again, it's best if people could use the microphone. Everybody's sort of spread out in the room. Okay, great. Thank you very much.
Video Summary
In this video, Dr. Ibaki from Algeria discusses the challenges faced by developing countries in the field of pediatric neurosurgery, specifically focusing on medulloblastomas. He talks about the heterogeneity of response to treatment and the need for better risk stratification based on molecular biology. He highlights the importance of advances in molecular biology in understanding the different subgroups of medulloblastomas and their implications for prognosis and treatment. Dr. Ibaki also discusses the limitations in access to advanced technologies, such as immunohistochemistry and fish analysis, in developing countries. He emphasizes the need for support from developed countries and investment in molecular biology to improve outcomes for patients in developing countries.
Asset Caption
Souad Bakhti, MD (Algeria)
Keywords
pediatric neurosurgery
medulloblastomas
molecular biology
developing countries
outcomes
×
Please select your language
1
English