Friday, February 6, 2009

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!


Anonymous said...

2 thumbs up! I like your style.

Keep on sharing information. It's helpful to everyone.

Anonymous said...

Good, important information presented in a way that is not giving specific advice.

Keep it up!

Dawn said...

I think it was brilliant! I also think your unwritten rules needs to be put in to book form. Keep them coming!!!

Zeynep Erden Bayazit said...

Couldn't help commenting. It is a funny coincidence of your entry, as we came out of surgery two days ago, hopefully with a complete resection. This has been the highest point for us so far. And you are absolutely right about having second opinions, looking for the best of the best. And may I add, still questioning and researching.

You have been a wonderful source of information for us, I surely check what you have on internet before and around any decision that we have to make about our daughter's treatment.

So, yes, keep up sharing information!

Anonymous said...

When it's your child and every decision seems extraordinary, I concur that the biggest is the surgeon. After 6 rounds of chemo we were introduced to the surgeon. World class expert in pediatric heart lung transplants. He emphasized how difficult the operation was going to be. We asked how many neuroblstoma resections he had done and the answer was five. After some more research we concluded that there are some surgeon who do that many in a week. We signed up for an extra round of chemo, arranged travel to NYC and prepared for an extended stay at the Ronald's house in Manhattan. MSKCC welcomed us, and there is great comfort when a surgeon that has performed thousands of surgeries specific to this condition says "I removed all of the tumor I could see and feel"...

Anonymous said...

hi i need a second opinion on my sons surgery,he was due to have his op on 26th of this month it was booked 2 months ago and the surgeon agreed to do it.Tody however they have cancelled my sons appointment and sed surgery is too risky for him,surely its more risky to leave the tumour in.He has stage 4 high risk nb and responded well to rapid cojec his tumour shrunk by 70% so i cant actually see how much more they evpected please e mail me a list of surgeon to contact im takin ur advice im running for that second opinion

Anonymous said...

Can I have a list of surgeons--

Thank you,

Michiko Lindsey said...

Your timing is impeccable. I just had Monday's surgery cancelled for my daughter in order to pursue a second opinion with Dr. LaQuaglia at Sloan Kettering. My ex-husband had complete faith in the local surgeon in San Diego, but if you can have the surgery done by the world expert, you would be crazy not to do it with him.

Mom to Kimiko Lindsey-Schroder, dx NB4 9/21/06

Krista said...

Your comparison with golf was right on! I have been having a hard time explaining myself to others but that is perfect! My son's surgery is scheduled for Thursday but I had a bad feeling about the surgeon when speaking with him today and am going to seek a second opinion in NYC. Our current Dr was concerned the NYC surgeon may be too aggressive around the blood vessel... but I feel that they also want us to stay with theirs.

Anonymous said...

Would love if u could send me the list of Dr. My 2 yo was dx 10/2012 NB4 with no primary tumor. We are in WV. Thanks. Christie

Anonymous said...

Can u send me a list my 3 month old daughter was diagnosed 3 weeks ago and chemo is taking a toll on her

beboyherrera said...

Thank you for this. Can you send me a list.

Herbert :

Anonymous said...

I appreciate the advice. Plain, simple and powerful. Can you please send me a list.

Anonymous said...

Hi Mark,
Well written. I too, need a list, but more than anything Id like to see what your opinion of that "one" surgeon in particular is, or rather..."who" it is? Hope to hear from you soon. My son was diagnosed with stage 4 HR NB about 2-3 weeks ago, just finished round 1 of chemo, out tumor is baseball sized in the chest behind heart, pushing on left lung and trachea, has spread into spine, chest bone, pelvic bones, marrow and femurs. It wasnt there in April so it showed its ugly face and grew in just 4 months. We are currently at Dallas Childrens and he just did a biopsy, MIBG scan etc then round 1 of chemo. Im also doing alternative treatments, diets etc. Hes trach and vent dependent with full colonic Hirshsprungs disease, CCHS and Epilepsy so lots other stuff to knock us down :-( Im a research guru and wont stop till I find the best! I hope your family is well, and I hope to hear from you!

Bre Sessions said...

I would also be curious to hear what surgeons you would suggest. My daughter's cancer did not respond to chemo and we are trying to determine where to go and who to go to for surgery. So hard to research this topic, and figure out what is best.

Ryan said...

Please send list and the "best". Thx

Anonymous said...

Please help me find the best doctors to Save our BabyGirl Rosannah (name of Facebook and go fund me page. She is in Australia and has a small soccer ball size tumor that is not responding to chemo and has been advised by a NYC doctor that surgery is too risky but no alternatives are give. Please tell me who the best is and if you have any ideas how to help get her to the US. Her family is desperate for help. Please check out the story on Facebook and share it with everyone. Please advise if you can
Thanks Mandy

Anonymous said...

Would love a list of surgeons. We are working with CHOC in orange county, CHLA and Sloan Kettering. We have a difference of opinion in 2 of them and are awaiting the 3rd. Please send to

Thank you!

Anonymous said...

Please send me your current best surgeon list! Need it for the NYC and PA area. Thank you for this valuable post and info!

Unknown said...

Could you please send me the list? Amazing article, thank you for writing it.

Unknown said...

Could you please send me the list? Amazing article, thank you for writing it.

Anonymous said...

Fantastic article,ouf daughter has hr nb she responded very well to induction chemotherapy, had surgery which removed approximately 80% of the tumor, she has had three cycles of immunotherapy treatment, however on a recent ct scan it shows that residual tumor has grown we are about to start chemotherapy, then hopefully surgery to take it out ,if the uk surgeons say they can't we will be seeking advice from elsewhere could you therefore please forward me the top neroblastoma surgeons, to thus address, I would really appreciate any additional advice