Journal of Pediatric Surgery Article

Preface by Beth K. Orgel, RN

Dear DSRCT Families and friends:

As a few of you may know, Dr Michael LaQuaglia, along with his peers at MSKCC, has recently published a new article on DSRCT. It is in a recent issue of The Journal of Pediatric Surgery. Dr LaQuaglia originally presented his findings at a Medical Conference in May of 2004. You can access the article in it's entirety, via a link at the end of my note. However, I would like all of you to read this message first.

The article as it is written, is grim. DSRCT is a rare, aggressive disease, but you all know that by now. That said, there is more to be noted. This article is accurate, as a HISTORICAL perspective, but therein lies some weaknesses in the authors' conclusions. I pulled this article apart, along with assistance from two MD's whom I know and trust.

First of all, the statistics and data used, date back to 1972. The analysis ends in 2003. In that time frame, MSKCC treated 66 people with DSRCT, my son among them. Although that number is probably many more than most institutions have seen, it is still small. In 31 years, it works out to little more than 2 cases a year. DSRCT was not even recognized as a distinct, separate form of sarcoma until 1981, 9 full years after these original statistics started being compiled.

Before 1981, DSRCT was treated with the standard protocols still in use now, for a wide variety of soft tissue sarcomas. 31 years ago, the P6 Protocol, and one full third of the drugs now in use, DID NOT EXIST. Surgeons did not know the significance between doing tumor debulking and the total tumor removal that Dr LaQuaglia recommends now. His conclusions are based on a goal that was not even recognized as important, at the time.

The article presents 2 different conclusions. One, dating back 31 years, DSRCT has an overall long term survival rate of 15%. The observation I made is that the statistical survival rate NOW, is much higher than that. If you base a new historical look back (which can't be done yet), of patients treated by the P6 Protocol and include total tumor removal, over the last (say 10) years?, I'll bet those numbers would be much different. The reason the article doesn't start from 10 years ago, is because the number of treated cases (2 times 10) 20 give or take, is not enough to draw any conclusions from, and could not be published with any degree of authority.

One section of the article notes 4 long term survivors, from 5+ to 11+ years. That's the 15%, those 4 of 66. If you START compiling statistical survival with the child now 11 years out and go forward, those numbers will immediately go up. One thing to note from the graphs in the article is that survival levels out at 4 years after diagnosis. If you make it that far?...... Greg is almost there. He's AT 3 1/2 years now. I refuse to believe that he's a fluke. His oncologist doesn't think so either. We came to the conclusion that all the kids who received treatment similar to his, including all the ones treated after him, from 2001-2005. will push that number way up.

We just don't know it yet, because it hasn't been long enough. We aren't 5 years out.

The second conclusion that was reached, is this:  You can use any protocol available, do radiation or not, do a stem-cell transplant or not. The biggest and most statistically significance difference in survival, depends on surgical skill. Those that had a 90% tumor removal or higher, are the ones that have a better chance. The survival rate at 3-5 years after complete tumor removal stands now at 29%. Remember, 3-5 years in this article covers 1998-2003. These are the kids, from 1998 and on, who will change the conclusions reached, the next time Dr LaQuaglia or Dr Kushner publish a historical perspective like this one.

With hope for the future, Beth

Journal of Pediatric Surgery Article (Full Text)