So Many Terms, So Much to Learn … |
An ESUN Editorial
Finding out that you, a loved one, or a friend has sarcoma cancer can be a frightening and stressful experience. All sorts of questions immediately spring to mind. "Do people survive this type of cancer?" "Will I die?" "What will happen to my family?" There is a new language to learn: CT scan, MRI, bone scan, pathology report, first-line therapy, neoadjuvant therapy, chemotherapy, side effects, dosing, radiation therapy, stable disease, progressive disease, palliative care, and metastasis. The list of challenges seems endless, and you are not the only one who feels this way!
Cures are treatments that eradicate an illness permanently. Many sarcomas are curable, particularly (a) low grade malignancies which only require surgery and (b) the pediatric sarcomas. Sarcoma is not one disease but rather a large group of different diseases. It is very important for your physicians to know the specific type of sarcoma you have, as the potential treatments are sarcoma-specific, and there may be a variety of treatments that can control the disease and extend life. The determination of which sarcoma a patient is diagnosed with should be done by a skilled pathologist with extensive background in sarcoma pathology. There is ongoing research into employing molecular biological methods in sarcoma pathology.
If you have been diagnosed with sarcoma, your medical team will discuss treatment options for your first-line therapy with you. Treatment may include a combination of local control (aimed at the primary tumor) and systemic therapy (designed to prevent or treat metastatic disease). Metastatic disease is disease that has spread from the primary tumor to other parts of the body. Some sarcomas have a low enough risk of metastasis that systemic therapy is not necessary and patients only get local control (e.g., surgery or radiation therapy). Even these patients sometimes get chemotherapy, aimed at making local control easier by shrinking the primary tumor.
First-line therapy is also called first-line treatment, primary therapy, primary treatment or induction therapy and it may consist of surgery, chemotherapy, targeted therapy, radiation therapy or some combination of these - all depending on the size, location, and stage of the tumor, as well as the specific type of sarcoma. Some patients receive neoadjuvant therapy in addition to first-line therapy. Neoadjuvant therapy is treatment that is given before the primary local-control treatment in order to shrink a tumor and control any subclinical distant tumor spread. As an example, neoadjuvant therapy could be the administration of chemotherapy before surgery is performed.
If your first-line treatment is surgery, the pathology report will determine if you need any further therapy. Often, none is required. In any case, when your first-line treatment is completed, you will undergo examinations and tests to determine how the cancer responded to the treatment. You could be told that you have had a complete response or a partial response, or that you have stable disease or progressive disease.
- A complete response means there is no evidence of disease (NED) on the examination, scans or other tests. A complete response is also knows as remission.
- A partial response means that some of the cancer has remained in the body, but there has been a decrease in size or number of areas of abnormal tissue (lesions).
- Stable disease means that the cancer is essentially unchanged in the size and number of tumors.
- Progressive disease means that the tumor has increased in size and/or in number.
Complete response, in some cases, means a cure. In other cases, patients with a complete response receive additional treatment. If you have a partial response or stable or progressive disease, additional therapy may be recommended. Adjuvant therapy consists of treatment that is given after the first-line treatment to lower the risk that the cancer will come back (e.g., brief high-dose chemotherapy). Progressive disease implies that the treatment was not effective and that additional treatments may be necessary to try to control the sarcoma. Second-line therapy is treatment that is given if the first-line therapy does not work or if it stops working.
Each person's sarcoma responds differently to treatment. Some sarcomas are slower growing than others, while some are extremely aggressive and very difficult to kill. Some people may experience side effects when being treated. There are problems that occur when the treatment affects healthy tissues or organs. For example, some of the most common side effects of chemotherapy treatment are: fatigue, pain, nausea, vomiting, decreased blood cell counts, hair loss, and mouth sores. Some treatments may produce long-term side effects as well.
Sometimes a residual amount of the cancer exists after treatment that cannot be detected by the post-treatment scans and tests. A recurrence (a.k.a., "relapse") means that the sarcoma has returned after a period of time where it was not detected. Thus, a "complete response" does not mean the cancer has been "cured" as it may recur later. If you have had a complete or partial response to your first- or second-line treatment, your medical team may recommend that you begin maintenance therapy to help lower the risk of the cancer coming back. Maintenance therapy also may be used for patients with an advanced cancer that has no cure to help keep the cancer from growing and spreading further. Maintenance therapy is not new in treating cancer, and its use in sarcoma may be growing. New clinical trial results (e.g., the SUCCEED trial and PALETTE trial reported on at ASCO earlier this month) show that maintenance drugs can help some patients with sarcoma delay a recurrence of their disease (i.e., the time to progression, a.k.a., the progression free survival time). Maintenance therapy may consist of a low-dose protracted course of therapy.
If sarcoma is extensive, growing, or if it has metastasized, the medical team may suggest that you enter a clinical trial if, to the best of their knowledge, no known first-line or second-line treatments will help. A clinical trial is a well-planned research study that tests a drug or treatment to see how well it works on people. Clinical trials may involve a variety of different approaches in dealing with cancer, such as using new drugs, using new combinations of existing drugs, using new protocols for administering drugs (for example: different dosing and/or cycle lengths), using new combinations of chemotherapy and radiation therapy, or employing entirely new methods for dealing with cancer. Clinical trials offerings do not mean that first-line or second-line treatments will not help; they mean that what is being offered has a research component. Clinical trials involve rigorous testing, reporting and adherence to specific guidelines. Not all patients are eligible to be involved in a clinical trial, even if it includes the specific cancer they have. There are constraints that might make you ineligible to participate in a specific clinical trial.
- A Phase 1 trial is intended to demonstrate the safety of a new therapy or combination of therapies.
- A Phase 2 trial, conducted after the Phase 1 trial, is intended to obtain information on the efficacy of the new agent or treatment regimen.
- In a Phase 3 trial, conducted after the Phase 2 trial, a randomized comparison is being made between a treatment that is considered standard and a treatment which is hypothesized to be superior to that standard treatment (e.g., "standard of care" and "standard of care plus a new agent"). Patients in Phase 3 studies may or may not have metastases.
Some patients with extremely aggressive and widely metastasized tumors may no longer have treatment options available to them, and hospice care may be recommended. Hospice and palliative care focus on relieving and preventing the suffering of patients. Palliative medicine is appropriate for patients in all disease stages, including those undergoing curative treatment as well as patients who are nearing the end of life. Hospice provides special care for people who are near the end of life and support for their families. Hospice care can be provided at home, in a special facility, or in a hospital setting. Both palliative care and hospice care employ a multidisciplinary approach to patient care, relying on input from physicians, nurses, chaplains, social workers, psychologists, and other health professionals in formulating a plan of care to help the patient and family.
The journey with sarcoma isn’t easy. There are so many terms and so much to learn. To assist readers in learning more about these and a myriad of other related issues, we have taken many of the articles that have appeared in ESUN and organized them in a Sarcoma Learning Center. Explore articles listed under the "Diagnosis & Treatment, Types of Sarcoma, Clinical Trials in Sarcoma, Sarcoma Questions & Topics, Sarcoma Video Series, and Additional Resources tabs and let us know what additional topics you would like to see covered.
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