The First Unwritten Rule of Neuroblastoma - Surgery

First, a disclaimer, I am a dad. I am not a medical professional. Verify everything I say with a medical professional who is an expert in neuroblastoma. Second, if your oncologist tells you I am wrong, ask another one - ask a neuroblastoma expert.

The First Unwritten Rule of Neuroblastoma is regarding complete surgical resection. This is a tough subject. The leaders of the neuroblastoma community are split on this topic. Although there is a sizable body of research on this topic there is no definitive answer in literature. Furthermore, if you chose to read the literature on this topic, be skeptical. Just when you think you understand it, you will realize that the opposite is also true. Both sides of this issue are right. Finally, I am not advocating radical surgeries that put children's lives in more risk.

I have a safer plan.

First, there are two really incredibly well-respected big neuroblastoma brains that are at odds on this topic. I deeply respect both of them and I honestly believe they are both right. These are Dr. Sue Cohn and Dr. Mike LaQuaglia. For the sake of argument, I believe that Dr. Sue Cohn would probably disagree with some of what I have to say. I have incredibly deep respect and admiration for her.

Technically, she is smarter than me and, frankly, she is much better dressed as well.

Let's begin.

My rule is: If your surgeon does not have a strong level of confidence at being able to completely remove your child's tumor, do not walk, run to get a second opinion from a surgeon that is respected as the best in the field of neuroblastoma.

Wow, I could just feel about 20 of my favorite oncologists cringe at that statement. In fact, before I even began to state why I believed this to be true I could already feel my inbox starting to fill up with nastygrams. Just wait, let me finish.

Well meaning physicians do not like statements like this for many reasons. First, they fear that parents will be taking more risks than necessary. They fear that statements like this will raise the incidence of radical procedures that put kids lives in jeopardy. They also fear for those that can not afford to get a second opinion or go to another institution. What affect do my words have on them?

They are right. I have the same fears.

Let's be straight! I am not advocating radical nephrectomies or anything of the like. I am advocating that, if you find yourself in this position, you talk to the best of the best. Period.

You may think that I am saying all of these things because I believe that a complete resection has a higher degree of survival than an incomplete resection. That is not my point. Actually, it is not even my reasoning. Let me explain.

If you look at the research over the years there is actually conflicting data on this subject. Some studies show that complete resections have impact in certain subsets (different stages) of patients and others do not. Personally, I believe that less disease is better than more. I believe that a complete resection has a better survival than an incomplete resection. I can say that the majority of the research bears this fruit out. In fact, Sue Cohn's article in the January, 2006 edition of "The Journal Of Pediatric Surgery" even supports this theory. However, the article's rationale behind the claim that children with complete resections have better survival than those with incomplete resections is smack on. In simple terms the argument is this: the kids that had complete resections had better survival because their disease was less aggressive. They could be completely resected because the disease was not as bad. They proved (small numbers, nothing is truly proven) that the kids that had complete resections had fewer local recurrences. The real determining factor in survival, though, was metastatic relapse (relapse in other parts of the body) not local relapse. However, one could also argue that the presence of local tumor would probably increase the risk of metastatic relapse. Regardless, I think we can see that there are many sides to the issue.

See, it is murky. Yes, it is true. Kids with complete resections tend to do better. However, were they destined to do better in the first place because their disease was less metastatic in nature? Quite possibly! I don't know the answer and this is the reason that my argument for getting a second opinion is not based on this survival statistic.

My argument is based on reality. It is based less on the resection itself and more on what follows. You see, resection is a key decision point in treatment. In many ways it dictates destiny, not necessarily survival destiny although that is a factor. You see, when you don't have a complete resection it complicates everything that follows and more often than not will lead to more toxic therapy that is probably less likely to produce the desired result - a happy, well- balanced and ALIVE 40 year old.

Think about it. Once you have an incomplete resection everything changes. Will a transplant do any good with bulk tumor present? Will it be as effective? Will the radiation be enough to get deep enough to reach far enough to kill every cell. Is every cell in the tumor susceptible to radiation and chemotherapy? What happens when you finish transplant and you still have disease present? You probably will. Is Accutane enough? Do you need MIBG therapy? Post transplant surgery? What consolidates you then?

You see, there are mountains of questions and complications.

Surgery is one decision point you want to get right.

So, yes, I know I have simplified it and generalized some things. Who would want to have an incomplete resection when you could have a complete one.

"I mean, come on, Mark. Just because we want one doesn't mean we get one. We don't get one because we don't have the option."

Wrong! The one thing we have is options. We may not like the options but we do have them. Furthermore, we can all agree that some surgeons are definitely better than others. Some have different styles, some are more aggressive, some are less, and some are simply gifted.

I am a good golfer. I really am. I am impressive. I even played professionally. Now, if we you were going to play in a tournament for $1 million who would you want on your team. Me or Tiger Woods? Tiger Woods, of course! Would it matter if you needed one of us to make a 1 inch put. Probably, not! Either of us could do it with equal prowess. He may be more stylish? He might be more funny? He might even be better to look at. I would probably try harder? But, we would both get the job done. But, if we were on the 18th fairway and all of the money was on the line, there was a gusty breeze and the green was sloping treacherously down towards the water; who would you want on your team?

You had better say "Tiger Woods" or I can't help you.

When the game is on the line, when there is question (any question) get Tiger Woods on your team.

Now, let's talk about what I have seen. I have seen hundreds of kids have incomplete resections that could have had a complete resection had they been in the hands of the right surgeon. I know that sounds arguable but I can't tell you how many second surgeries I have seen (performed by a second surgeon) that produced a complete resection and it had nothing to do with the fact that the tumor "become" easier to resect. I have seen many kids with incomplete resections have local relapses that later had to be removed (and this time by a surgeon that was capable of getting the job done.) I have seen them endure multiple surgeries, mountains of chemo, radiation, and a slew of medications and treatments that put their lives in jeopardy. Where, had they looked elsewhere - had they gotten that second opinion - they probably would have had to only accept a small amount of risk to avoid a relapse and a mountain of toxicity. To be fair, I have also seen the other side. I have seen radical surgeries that have left kids in pretty bad shape for weeks and sometimes months. But, when the experts have been in the room I can honestly say that regardless the cost, most parents still came out with a sigh of relief.

Ask the question! Don't believe any one surgeon. It is too important of a decision. It changes the game. It impacts every decision made from that point on. If you don't do this you will always doubt your decision. You will always wonder. This is the number one regret I hear from other parents.

I offer no guarantee that the best surgeon in the world will be able to resect your child's tumor. I offer no guarantee that the risk of surgical complication will be worth the increased risk of relapse. I have no idea whether the risk of a more radical surgery is as bad as the toxicities from further treatments.

I don't know.

That is your decision.

But, you don't know either. You don't know, until you go ask.

It isn't just the surgery. It impacts everything.

Go do it. Go advocate for your child. Go get the second surgical opinion.

For those interested in finding the very best neuroblastoma surgeons in the world you can find them all over the Internet - one in particular. You can also write me and I will give you a list. However, I will not state them here because I don't want this to turn into advertising for a particular surgeon or group of surgeons. That is not the point.

If your child has a tumor that is not believed to be completely resectable go to the best. Get a second opinion.

There you go, purpose - without any medical advice. How did I do? Let's hear your thoughts!