This is not meant to be a substitute for medical advice. Please discuss all issues with your doctor. The following is information gathered through patient experience of dealing with this disease.
It is highly recommended that if you have been diagnosed with desmoplastic small round cell tumor to seek out a sarcoma specialist or go to a Sarcoma Centers or Sarcoma Program for treatment options.
What to do
As difficult as this is to hear it is essential that you find out what stage and grade of tumor you are dealing with. The reason that stage is important is because it will determine what treatment options are available for this disease at that stage.
A staging system determines how much the cancer has spread and what grade the tumor is (how abnormal it looks under a microscope). The following common system is used to stage soft tissue sarcoma. You will see these terms used on your medical records:
- T stands for the size of the tumor.
- N stands for spread to the lympth node.
- M stands for metastasis (spread to distant organs).
- G refers to the histologic grade of the tumor (what it looks like under a microscope).
- T1: The sarcoma is less than 5 cm (2 inches)
- T2: The sarcoma is 5 cm or greater
- a: The tumor is superficial – near the surface of the body
- b: The tumor is deep in the limb or abdomen
- N0: No lymph nodes have sarcoma cells in them
- N1: Lymph nodes are present that have sarcoma cells in them
- M0: No distant metastases (spread) of sarcoma are found
- M1: Distant metastases of sarcoma are found
- G1: Looks like normal tissue – tends to grow slowly
- G2: Looks less like normal tissue – faster growing
- G3: Only slightly looks like normal tissue – even faster growing
- G4: Does not look at all like normal tissue – fastest growing
Stages for Soft Tissue Sarcomas Stage I: G1-2, T1a,b, T2a,b, N0, M0
- The cancer does not look too different from normal tissue. The tumor can be any size, but it has not spread to lymph nodes or more distant sites.
Stage II: G3-4, T1a-b, T2a, N0, M0 The cancer looks very different from normal tissue and may contain many dividing cells. The tumor can be any size. If it is less than 5 cm (2 inches) it can be superficial or deep. If it is larger, then it must be superficial. It has not spread to lymph nodes or more distant sites.
Stage III: G3-4, T2b, N0, M0 The cancer looks very different from normal tissue and may contain many dividing cells. It is larger than 5 cm (2 inches) and is deep. It has not spread to lymph nodes or more distant sites.
Stage IV: Any G, Any T, N1, M0 or Any G, Any T, Any N, M1 The tumor can be any size and any appearance, but it has spread to lymph nodes near the tumor or to distant sites.
It is suggested that you do not focus on statistics or prognosis. Everyone is an individual. There is hope and survivors from each stage of cancer.
Choosing an Oncologist
The most important issue in choosing an oncologist is to find someone who is reasonably informed about DSRCT and sarcoma. This is an extremely rare disease and there are many oncologists who have never treated or even seen this disease before. Many former patients and caregivers highly recommend that DSRCT be treated by a sarcoma specialist at a Sarcoma Center. Adding to the rarity of the disease is that DSRCT is often considered a childhood disease. It is far more likely that a pediatric oncologist is more familiar with DSRCT than an adult oriented oncologist. Patients should keep this in mind when selecting an oncologist. Sarcoma is a rare and difficult disease to treat. Patients should at least make sure that their oncologist consults with a sarcoma specialist at some point about their treatment. Once a treatment plan has been decided from a sarcoma specialist it is often possible to be treated locally using your own oncologist.
At some point you may want to seek out a second or third opinion about your treatment options. This is a rare disease and your doctor may not be aware of new or promising treatments. Doctors may also assume or tell you that the tumors are inoperable . . always get a second opinion or third if a doctor tells you this. What may be inoperable for one surgeon, may not be inoperable for another. This disease has a poor reputation among regular oncologists and the news they give their patients is grim. But, there are survivors and that is what you need to focus on at all times.
Far more important than seeking out the 'best' oncologist to handle your treatment is finding someone who is willing to fight with you for as long as it takes. Someone who will invest their time in you and try to locate the very best treatment possible even if that means sending you to another specialist.
Staging information from the American Cancer Society