I'm done with chemo. I'm 34 days away from surgery at Sloan Kettering. I have no medical appointments related to the liposarcoma until I start my pre-op appointments at Sloan Kettering on 3/8, with surgery on 3/10. I am finally feeling human again after the last round of chemo, although my taste buds are still way off, and I'm dealing with bone pain from the Neulasta injection on Monday. But, those are minor issues compared to the big picture. I thought I'd take this break to explain in more detail what I'm facing over the next weeks, months and years. Maybe this post will be useful to patients newly diagnosed with liposarcoma in the future, and for now, it will explain to my friends a bit more about my prognosis.
Sarcomas, in general, make up about 1% of all cancers. There are approximately 85 subtypes of sarcoma, and even though they are all linked together, they are many very different diseases. This quote, from a recent sarcoma conference, sums it up pretty well:
"I also think that these studies show, especially the trabectedin/eribulin studies, show very nicely that we can parse out some subtypes of sarcoma. I’d like to see that go even further because lumping leiomyosarcoma and liposarcoma in a study together is still putting breast cancer and colon cancer together in a study."
Essentially, the doctor is saying that in a clinical trial where liposarcoma and leiomyosarcoma were studied together, it was somewhat absurd, because they are radically different diseases. And, to the best of my knowledge, these two sarcomas are considered "similar" in many respects. The drug trabectedin that is mentioned above has been used in much of the world to treat both of these sarcomas and it was recently approved for both in the US. The other drug, eribulin, is a drug that has been used for breast cancer, and it showed recent promise in clinical trials for just liposarcoma. To the best of my knowledge, its recent approval for advanced liposarcoma makes it the first drug ever approved in the US where the approval listed only liposarcoma.
So, there are 85 subtypes, most are very rare, and they really are very different diseases. Liposarcoma is one of the more common sarcomas. I've read that it's the most common, and alternatively, I've read that another sarcoma is the most common. I think that somewhere around 15% of all sarcomas are liposarcomas.
But, it doesn't end there. Liposarcoma has its own subtypes and it also occurs in very different body parts, and the prognosis and treatment vary a lot based on where the cancer starts. The four most common subtypes are well-differentiated, myxoid, dedifferentiated and pleomorphic. There are then variations beyond this. There is a mixed diagnosis, where multiple cell types are found. Within myxoid, there is a subtype called "round cell variation" as well. Additionally, many dedifferentiated tumors start as well-differentiated, but over time, the cells morph, because the cancer is rarely found early. That is what happened to me. In general, well-differentiated cancer is the easiest to cure, pleomorphic is probably the worst diagnosis, and the other two are pretty bad as well.
I believe that liposarcoma occurs most frequently in the extremities - arms and legs. The tumors can be hard to detect until they are very large, and surgical excision is difficult, often resulting in the loss of lots of muscle tissue to get clean surgical margins. Amputations are not uncommon, to be sure that the tumor is fully excised. After the extremities, the most common location is the retroperitoneal space. Think "close to the the kidneys". That is where my tumor was located.
So, my final diagnosis, after my original surgery, was retroperitoneal dedifferentiated liposarcoma. I think there are a few hundred such diagnoses made in the US each year. Part of the problem with a diagnosis like this is the lack of doctors who really understand the disease. My surgery last September was done by a urologist. We didn't know exactly what the tumor was before surgery. There were some indications that it was kidney cancer, but two different doctors, including my surgeon, had their doubts, because the CT scan didn't look "right" for standard kidney cancer. But, because the tumor was so large that it was causing my right lung to collapse, it had to come out. There wasn't time to do a biopsy, get the diagnosis, and then consider the options.
If my primary care provider had tested my blood or done some imaging earlier, we might have had that option. But, he thought my tiredness was due to stress, and didn't even find that I had anemia until months after I'd reported symptoms to him. Then, I saw a hematologist to determine the cause of the anemia, but he never ordered any imaging either, despite my reports of diffuse abdominal pain. All this time, the primary tumor was getting bigger and bigger. I had no imaging until the tumor got large enough to partially collapse my lung, and then the imaging was only ordered after I went to the emergency room.
When I received my post-op pathology report, it was obvious that there were not clear surgical margins.
Liposarcoma is a cancer that tends to recur. And recur. My oncologist has told me that there are two distinct styles to treating the disease. On the east coast (think Dana Farber, Mass General, Sloan Kettering and Johns Hopkins), the standard is to treat with surgery every time the disease recurs. For well differentiated types, this can lead to a cure. Or infrequent surgeries. But, it tends to keep coming back, and eventually, due to scar tissue or the cancer moving into an area that can't be resected, surgery is no longer an option. At that point, chemo and radiation become the options, although neither work great, in general, for liposarcoma.
On the west coast, (MD Anderson, UCLA, and others I'm forgetting now), the treatment tends to be multi-modal - use everything right up front - chemo, radiation, and surgery. Well, this might be a gross generalization, but it's how it was explained to me. My oncologist spent a number of years at MD Anderson and has worked with sarcomas extensively. It is only because of her prior experience in this area that I've let her direct my treatment, rather than going directly to one of the Boston or NYC facilities for all of my treatment.
In my case, because my tumor was dedifferentiated, the oncologist thought that my risk of recurrence was very high. I've already had the primary tumor removed (in September) and I had a recurrence (or growth of cells missed in the primary surgery) by November. My oncologist said that we could try the surgical route for the three new lesions that showed up in November, but she believed there was zero chance for a cure with this option. She suggested chemo, surgery and possibly radiation as the treatment. Given her experience, and an interaction with a local surgical oncologist that I didn't particularly like, I opted for her approach.
So, my chemo is done. Surgery is coming up. The surgery will be done by one of the top liposarcoma surgeons in the US, if not the world. There might be some intra-operative radiation as well. It depends on the CT scan that will be done 2 days before surgery.
So, what happens after the surgery? What is my future going to be? Remember that there may or may not be radiation, but I'm not going to focus on that now.
Approximately 3 months after surgery, I will have a CT scan. That scan could come back as NED (No Evidence of Disease) or the cancer might have returned. As long as I'm NED, the scans will continue quarterly, probably for 2-3 years, and then drop over time to every six months and eventually, a year. The odds of a complete cure, to be honest, are pretty low. Dedifferentiated liposarcomas are sometimes cured, but the rate is low. The 5 year survival rate for my diagnosis (including some other details about tumor size, mitotic rate, etc.) is about 50-50.
It's a funny survival curve though. It has a steep early decline and then a long downward sloping tail. Many people are lucky to get 12-18 months. With the new chemo drugs, I'm guessing that this will change to something more like 18-36 months over time, but in the worst case, the disease recurs over and over again, inoperability happens, and then you buy as much time as you can with chemo. In some cases, each recurrence becomes more aggressive and organs (the lungs, liver, pancreas, etc.) are attacked, and the timeline and treatment options are grim.
My oncologist has said that a cure is possible, but I'd bet the chances are less than 10%. Her real goal is to change the nature of the disease for me. She wants to get rid of the dedifferentiated subtype, and hope that recurrences are infrequent and well-differentiated. If this occurs, it essentially slows the timeline and gets me onto the early part of the tail of that curve. Well-differentiated tumors are treated by surgery primarily, because they don't respond well to radiation or chemo. So, the real goal right now is to use the non-surgical modalities to kill any peripheral dedifferentiated cells, and then hope the future recurrences, if they happen, are well differentiated - a less aggressive cancer. If we can achieve that, the 5 year mark becomes realistic. Ten years may be possible. Maybe more.
So, I'd guess there's maybe a 10% chance for a cure. Maybe a 30%-40% chance that the doctors can change the nature of the disease and extend me past that five year mark. And, if I see frequent and early recurrences, I'll fall into the 50% that don't (or is it doesn't? Is "50%" here singular or plural?) see 5 years. In that latter case, I will have to weigh options about quantity vs. quality of life. I don't want to go through pointless treatments with a highly degraded quality of life.
But, some of the drugs that are available to me are due to previous patients, who participated in clinical trials. I don't look at clinical trials as a last gasp for me. I look at them as paying it forward - a chance to help future patients. There are many patients in the past who participated in trials that didn't help at all. Some participated in trials that did help. Someone has to be the guinea pig for these trials. Quality of life is important. But, so is advancing science. But, that chapter is for the future.
In the interim, I've got a life to live. I'll be on the treadmill at the gym tonight and I'll be out teaching skiing this weekend. After my surgery in a few weeks, I'll need some time to recover, but trout season is coming soon. And, if all goes well, I will meet my goal of 45 miles at the Ghost Train ultramarathon in October in NH.