Soft Tissue Sarcoma – One physician’s Viewpoint

by

Dr. Murray F. Brennan

Chairman, Department of Surgery

Memorial Sloan Kettering Cancer Center

One of the things that bother me as a physician, who cares for patients with soft tissue sarcoma, is the pessimistic and often imprecise information readily available on the internet. 

If I had a soft tissue sarcoma, I would like to go to a place where I would get a broad overview before I learned of all the difficulties. The information available often focuses on people who have developed metastasis, and who have undergone several treatments, but have not been cured, and on occasions have not been helped, despite aggressive regimens. The presumption, therefore, for the newly diagnosed sarcoma patient is that it is a terrible disease and that the diagnosis is a death sentence. 

In fact, the converse is true, the majority of patients who present with a soft tissue sarcoma are cured, and the majority of those are cured by their initial operation. More importantly, the risk factors for having a subsequent recurrence of the tumor, either in the same place or elsewhere, are now very well known.

I would like to know, when first confronted with the diagnosis, that the majority of patients are cured. I would then want to know “what is the likelihood I am in the cured group,” and if I am not, “what else can I do to improve my chances.” 

The outcome of patients with soft tissue sarcoma is highly dependent on the site at which the tumor develops. For example, a sarcoma that develops inside the abdomen has a greater risk of recurrence and a higher risk of the patient dying of sarcoma than a patient who has a tumor of one of the limbs.  Other risk factors have been clearly characterized; the size of the tumor is important (smaller is better), grade, i.e. low grade has a limited chance of spreading elsewhere (metastasis), but high grade lesions have a far greater risk of spreading elsewhere, and becoming life threatening. These factors do not act independently. The combination of large and high grade is much worse than the combination of large and low grade.  All of these factors are readily known, and we have made attempts to characterize this by developing ‘nomograms’ that predict the risk of dying of the tumor and the risk of having local recurrence.  We have made similar models to predict risk after a local recurrence. These are not specific and accurate for every patient, but are often more accurate than current staging systems, and are more reliable for the common sarcoma types, like liposarcoma and less reliable for the rarer subtypes.

While local recurrence is a fearful event for the patient and for the treating physician, on many occasions a local recurrence does not influence survival.  While the presence of a local recurrence at the site of the original tumor may suggest the possibility of finding tumor elsewhere, that is not necessarily so, and a patient can be cured after a recurrence at the initial site. 

Often all histologies (all types) of sarcoma are treated as if they all behaved the same. They do not. This is complicated by the fact that different kinds of sarcomas occur at different ages.  While rhabdomyosarcoma is predominantly a sarcoma of children, synovial sarcoma is a tumor of adolescents and young adults, and a liposarcoma occurs throughout adult life. The behavior of each and every sarcoma can be different depending on the patient’s age. 

Readers of this small piece may well say “this is all right for Dr. Brennan, he does not have a sarcoma,” but , my life has been to try to help patients with sarcoma, and when I see patients unnecessarily terrorized, I feel for them. 

Newsletters and websites that seek to educate can only, in the long-term, lead to a better educated population and better education translates into better understanding and better understanding translates into greater efforts to improving outcome.  Our obligation is to provide information that is accurate, realistic and where appropriate optimistic, not pessimistic.

V1N4 ESUN Copyright © 2004 Liddy Shriver Sarcoma Initiative.