Frequently asked questions about sarcoma, answered by Dr. Murray F. Brennan, former Chairman, Department of Surgery, Memorial Sloan-Kettering Cancer Center.
Is sarcoma cancer?
Yes. Sarcoma is a malignant tumor of the soft tissues of the body, such as fat, muscles, nerves, tendons, and blood and lymph vessels. (Soft tissue sarcomas are uncommon: according to the American Cancer Society, approximately 8,700 new cases of soft tissue sarcoma are diagnosed each year in adults and children in the United States.)
When the sarcoma starts in bone rather than soft tissue, it is called osteogenic sarcoma, or occasionally chondrosarcoma (cartilage sarcoma).
Are all sarcomas the same?
No. Soft tissue sarcoma covers a wide range of tumors. Some grow slowly; others are highly malignant tumors that can, at the time of first diagnosis, already have spread to other sites and may not be curable.
What kinds of people get sarcomas?
Sarcomas can affect anyone, from the young to the old. Different kinds of sarcomas occur at different ages. We do not know of any particular personality type that is at more risk of sarcoma than others.
What determines whether I have a "good" or "bad" sarcoma?
You will hear a lot about the "risk factors" for sarcoma. These are mainly:
- The location of the tumor
- The size of the tumor
- Whether or not it has spread to other parts of the body
- The special characteristics of the tumor that can only be determined after biopsy
The important term you will hear about in the pathologic analysis is "grade."
What is meant by "grade" for sarcomas?
Grade is a descriptive term provided by the pathologist who examines the tissue. Grade is a measure of how aggressive the tumor could become. Low-grade tumors usually stay confined to one place. High-grade tumors have the capacity (usually seen in up to half of large high-grade tumors) to spread elsewhere.
Where can soft tissue sarcomas spread?
It depends on where they start. In high-grade soft tissue tumors of the limbs, the most common site of spread is to the lung. Soft tissue sarcomas inside the abdomen can spread to the liver. Most experienced physicians who manage sarcoma will be able to tell you what your risks are, and will know where is the likely place to look for spread.
Am I going to die?
The majority of patients with soft tissue sarcoma can be cured. In fact, the five-year survival rate for people with soft tissue sarcomas is about 90 percent, if the cancer is found in the early stages. It should be noted too that treatments are always improving and often result in an even more favorable prognosis for patients recently diagnosed.
Is sarcoma painful?
No. Pain is uncommon in sarcoma unless it is very advanced or in special rare sites.
What are the important questions to ask my doctor before treatment?
Perhaps the most important thing is to be sure you are talking to a doctor who understands, and has experience with, the management of sarcoma. Because the disease is so rare, it is very important to get to a doctor who knows about the tumor. This is important not only to assure that you get the best treatment, but also to avoid getting more treatment than you need. Such sarcoma specialists usually work with a team, so if the primary sarcoma physician is an expert, you can expect he or she to have the best and most experienced pathologists and radiologists to help.
Is amputation ever needed for sarcoma?
Yes, but less and less commonly today. Twenty years ago amputation was virtually the only form of treatment for patients with sarcoma of the limbs. It is now required in fewer than 1 in 20 of all such patients.
Will I need chemotherapy?
Chemotherapy is usually given before an operation for very high-risk sarcomas and is also given after a tumor has spread.
Will I need radiation therapy?
Radiation therapy is commonly given to limit the risk of a local recurrence at the same place where the sarcoma was removed.
Are there different forms of radiation therapy?
Yes. Brachytherapy is the local application of radiation therapy through small tubes placed under the skin at the time of surgery. It usually lasts about four or five days. External beam radiation therapy is given for four to six weeks for a few minutes a day, five days out of seven.
When will I know if I am cured?
In soft tissue sarcoma, that is difficult to assess. In the majority (70 percent) of patients whose disease will recur, this will happen in the first two years. However, patients with sarcoma are usually followed for a minimum of ten years, as some patients can have a very late recurrence of their tumor. Such late recurrences are often more favorable, however, and can be readily and effectively treated.
Why is sarcoma so rare?
We really do not know.
Does sarcoma run in families?
Sarcoma can occur in families, but that, too, is very rare. Usually there is some predisposing disease that is known to lead to an increased frequency of sarcoma. If your doctor is familiar with sarcoma, he or she will be familiar with those diseases.
Can sarcomas be caused by chemicals?
We believe that, on rare occasions, sarcomas can be caused by environmental exposure at high level to some chemicals. This, too, however, is very rare.
Why am I told that sarcoma is hard to diagnose?
Because sarcoma is rare, very few doctors ever see a sarcoma in their lifetime. They do, however, often see benign lumps and bumps, so they may be unlikely to think of sarcoma.
Can injury cause sarcoma?
We believe this is very rare. More commonly, patients first notice a sarcoma when they bump a lump on their leg or on their arm.
Who should treat me?
The most important thing is that you see a doctor who is familiar with sarcoma. Whether that doctor is a surgical oncologist, an orthopaedic (bone) oncologist, a radiation oncologist, or a medical oncologist is probably less important than that the doctor is familiar with the disease. A doctor familiar with sarcoma can send you in the right direction for diagnosis and treatment, and aid in your rapid recovery.
How can my family help me?
As in the treatment of any cancer, the support of family and friends can be crucial. Do not be afraid to ask your friends and family to help. Often, the simplest thing is the most helpful: help me find the right doctor, please drive me to the hospital so I don't have to find parking, please look after my children and pets while I am away.
How can I help myself?
Educate yourself and learn to understand your disease. Ask questions of your medical team, and be an active partner. While it is certainly true
that the diagnosis of any cancer can be frightening, it is not the diagnosis or the label of cancer that hurts or kills us. It is important for your emotional well being that you not imagine the worst when the worst is not likely to happen. It may help to remember the adage, "Living with cancer is not a problem, dying is."
