KAKS Interview: Prof. Francis Munier, Lausanne, Switzerland
“Informing parents that their child has cancer is a huge challenge!”
Prof. Francis Munier has been a retinoblastoma specialist at the Hospital Ophtalmic Jules-Gonin in Lausanne, Switzerland, for more than 30 years and explains in this KAKS interview what parents need to know when their child is diagnosed with retinoblastoma.
How has retinoblastoma treatment evolved over the last 10 years?
There has been a change of attitude in the therapeutic approach to retinoblastoma due to the progressive switch from systemic (=intra-venous) to more targeted chemotherapy.
This evolution was made possible by the sequential introduction of new routes of drug delivery directly into the eye and its various compartments, namely the intra-arterial, intra-vitreal and intra-cameral chemotherapies.
This in turn has moved the frontiers of conservative management, expanding the indications for more advanced disease.
This trend is illustrated by a 90% decrease in the primary enucleation rate of unilateral IIRC group D retinoblastoma in Lausanne from >60% to 5%.
Another source of positive evolution is the implementation of new imaging tools in the operating theater, such as optic coherence tomography (OCT), which has allowed significant improvements in the conservative management by allowing earlier detection of relapse threatening either the optic head or the macula.
What are the biggest achievements from your point of view?
The complete eradication of external beam radiotherapy is certainly the biggest achievement, followed by the significant decrease in the use of systemic first line chemotherapy.
How important is it for parents to seek a second opinion when their child is diagnosed with RB?
I think that a second opinion should always be offered to parents whenever the indication for upfront (= primary) enucleation is debatable or challenged by the parents, since consensual criteria for primary enucleation are still unavailable.
This in contrast with secondary enucleation (after failure of conservative therapy), for which a position paper on behalf of the European Retinoblastoma Group was published in 2021, distinguishing absolute and relative indications.
A similar paper is expected in the near future regarding primary enucleation in order to make a distinction between relative and absolute indications to remove the eye.
This said, our role is also not to delay decision-making beyond 7 to 15 days.
How much influence can parents have on their child’s treatment and what crucial questions should they ask?
Most important is to follow the treatment planning and to take precautions against infectious diseases by limiting child’s exposure, especially during the initial phase of treatment.
The most crucial questions in my view are always related to life prognosis and the influence of treatment strategy on the risk of metastases.
What is your most precious advice to them?
First of all, I don’t recommend searching the internet, where a flow of unfiltered information can only lead to overwhelming confusion.
No doubt the diagnosis of retinoblastoma is heartbreaking news, but passed the initial shock of the announcement of eye cancer, parents need to know that the overall survival is exceptionally good compared to other pediatric cancers.
This remains true as long as the first line treatment, conservative or not, is not delayed.
Whenever conservative management is preferred, eye preservation is not only very high even in advanced and bilateral cases, but also associated with good vision in the vast majority of them in at least one eye.
In advanced retinoblastoma, it is important to inform the parents that the active phase of treatment can last up to a year, with monthly examinations and treatment under anesthesia.
Decision to stop therapy can be decided at any time without increased risk for metastasis, provided that subsequent surgery to remove the eye is not delayed.
How important is it to decide together as a team with pediatric oncologists?
The management of retinoblastoma is multidisciplinary in nature.
The decision-making should reflect this and involve the elaboration of a consensual strategy between pediatric and ocular oncologists.
It is normal that differences of opinion may occur among the team, but these should be resolved before talking to the parents.
Why can treatment suggestions be different in different centers?
The recruitment area of each center varies with local, national and international referral patterns stratifying centers into secondary, tertiary and quaternary levels of care.
These centers will differ from one another by the size and composition of the multidisciplinary team as well as the sophistication of their technological platform.
Another potential source of divergent opinions between centers comes from the collaboration mode between onco-pediatricians and ocular oncologists.
Is the preservation of the eye always to be prioritized or which other factors have to be considered in a decision?
The general trend in ocular oncology is clearly to be less mutilating, by keeping both eyes without jeopardizing the so-called “state of metastatic grace”.
In other words, preservation of the eye can be decided as long as survival is not compromised.
Have you experienced very unexpected outcomes (both positive and negative) in the treatment of small RB patients?
Which patients do you remember most?
Despite more than 30 years’ experience, I still witness paradoxical or counterintuitive responses to treatment, or unexpected tumor behavior.
I have had several instances of patients where an initial unfavorable response to treatment unexpectedly evolved into complete remission.
Inversely, I have seen patients with prolonged tumor activity, relapsing up to 6 years after the last treatment, but fortunately such observations are really exceptional.
Regarding the second question, my first thoughts go to patients I have treated from diagnosis and who later consulted for their own children, whether affected or not.
They survived their condition and founded their own family, thus continuing the cycle of life like any other person.
You treat young children with cancer, but somehow also always the whole family – is that more than an ophthalmologist usually has to do and how do you manage that?
The management of a patient affected with retinoblastoma and his or her family goes far beyond the role of an ocular oncologist.
The role of the whole team is crucial and each member contributes to the final outcome of the patient, whether successful or not.
Is that perhaps also what makes your job so special and fascinating?
There is an infinite variety in the circumstances, both familial and medical, in every child we treat.
Cancer is hard announcement to make, especially in a child, and the trust that is laid in us to provide the best possible outcome is a very precious gift.
It is why taking care of patients and families struggling against retinoblastoma cannot be compared to any other job, nor can it be limited to the search of a cure.
It is like embarking on a boat for a long-lasting journey, where the path to success depends not only on the experience of the captain and crew, but also on the active participation of the passengers and the quality of the relationship between all.
Anything you would like to say to parents whose child has just been diagnosed with retinoblastoma?
Firstly, I would congratulate the parents, on their recognition of an ocular problem and often perseverance in bringing their child to a specialized center.
In many cases, in particular for those not fortunate enough to be living in more highly developed countries, the path to finding the proper care for their child may be long and arduous.
Secondly, not to panic, to know that both life and eye survival is extremely high.
Thirdly, there may be a sense of culpability which should be vigorously discarded, since the parents are never at fault.
Now there may be a legitimate worry that future children may also develop the disease.
In this respect genetic analysis will be undertaken to determine any risk and provide help with the family planning.
GERMAN TRANSLATION:
“Informing parents that their child has cancer is a huge challenge!”
Prof. Francis Munier has been an RB specialist at the Hospital Ophtalmic Jules-Gonin in Lausanne, Switzerland, for over 30 years and explains in the KAKS interview what parents need to know when their child is diagnosed with RB.
When parents receive a diagnosis of RB for their child – what is your most valuable advice to them?
First of all, I don’t recommend searching the internet, where a flood of unfiltered information can lead to absolute confusion.
What are the biggest achievements from your point of view?
There is no doubt that a diagnosis of retinoblastoma is traumatic news, but after the initial shock, parents need to know that the overall survival rate is exceptionally good compared to other childhood cancers.
As long as the diagnosis and treatment are made in time, of course.
Parents need to know that eye preservation is now not only very high, even in advanced and bilateral RB cases, but also associated with good vision in at least one eye in the vast majority of cases.
In advanced retinoblastoma, it is important to inform parents that the active phase of treatment can last up to a year, with monthly examinations and treatments under anesthesia.
The decision to discontinue therapy can be made at any time without increasing the risk of metastasis, provided that subsequent surgery to remove the eye is not delayed.
How important is it to make decisions together as a team with pediatric oncologists?
The treatment of retinoblastoma is inherently multidisciplinary.
Decision making should reflect this and involve the development of a consensual strategy between pediatric and ocular oncologists.
It is normal that there may be differences of opinion within the team, but these should be resolved prior to discussion with parents.
Why can treatment suggestions be different in different centers?
RB centers differ from one another in terms of the size and composition of the multidisciplinary team and the technical and medical facilities available.
This results in different treatment options.
Another potential source of divergent opinions between centers arises from the nature of the collaboration between oncopediatricians and ocular oncologists.
So how important is it for parents to get a second opinion?
I think that parents should always be offered a second opinion if the indication for primary enucleation (= eye removal) exists or is questioned by the parents, as consensus criteria for primary enucleation are not yet available.
This is in contrast to secondary enucleation (after failure of conservative therapy), for which a position paper was published in 2021 on behalf of the European Retinoblastoma Group, in which a distinction is made between absolute and relative indications.
Very important: decision-making should not be delayed for more than 7 to 15 days.
Have you experienced very unexpected disease progressions when treating small RB patients?
Despite my more than 30 years of experience, I still experience incredible, unexpected courses of treatment.
I have had patients in whom an initial unfavorable response to treatment suddenly developed into a complete remission.
Conversely, I have seen patients with persistent tumor activity who fought for their eye for six years and still lost it, but fortunately such cases are truly the exception.
I particularly remember patients who I treated as children and who came back to me years later when they had children of their own.
They survived their illness, started their own families and now live like everyone else.
That makes me very happy.
Is that perhaps also what makes your work so special and fascinating?
Informing parents that their child has cancer is a huge challenge.
and the trust placed in me and us to achieve the best possible outcome is a very precious gift.
That’s why caring for patients and families struggling with retinoblastoma is unlike any other job.
Also because there is no one right treatment.
It’s like going on a long voyage – the road to success is long and depends not only on the experience of the captain and crew, but also on the active participation of the passengers and the quality of the relationships between everyone.
What would you like to say to parents whose child has just been diagnosed with retinoblastoma?
Since in the majority of cases it is the parents who discover the tumor in the eye, I would first like to congratulate them on recognizing an eye problem and then acting quickly.
An early diagnosis may not only save the eye but also the life of your child.
Secondly, I would like to tell parents not to panic, they need to know that the chances of survival for their child are extremely high.
Thirdly, I want the parents to know that they have done nothing wrong and that they are not to blame.
A retinoblastoma is a coincidence, a freak of nature and not the parents’ fault