Types of Treatment for Ewing Sarcoma

Executive Summary

Treatment recommendations depend upon the size, grade and type of tumor, metastasis, possible side effects, and patient’s preferences and overall health. The common treatment for Ewing sarcoma involves chemotherapy, surgery, radiation therapy, and stem cell transplantation or bone marrow transplantation. Systemic chemotherapy gets into the bloodstream to reach cancer cells in the body. Ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a swallowed pill. Children and young adults with Ewing sarcoma should receive doxorubicin (Adriamycin), cyclophosphamide (Cytoxan, Neosar), etoposide (Toposar, VePesid), ifosfamide (Ifex), or vincristine (Oncovin, Vincasar PFS). Radiation therapy uses high-energy X-rays to kill cancer cells. It is combined with chemotherapy during treatment. There are two types of stem cell transplantation depending on the source of the replacement blood stem cells – autologous (AUTO) and allogeneic (ALLO). Palliative care includes medication, nutritional changes, emotional and spiritual support and other relaxation therapies.

Treatment for Ewing Sarcoma

“Standard to care” refers to the best-known treatment. In cancer care, various doctors work together to bring out an overall treatment plan for the patient. This is called a multidisciplinary team. 

Treatments recommendations depend on many factors:

• The size, grade and type of tumour
• If the tumour has increased to other parts of the body
• Possible side effects
• The patient’s preferences and overall health 

Chemotherapy

Chemotherapy uses medicine to kill or stop the growth of cancerous cells. Depending upon the stage, different chemotherapy is given. The point that makes a difference is how the chemotherapy enters the body and which cells it affects.

A chemotherapy schedule usually consists of a certain number of cycles over a fixed period. A patient can be given one drug at a time or a combination of different drugs given simultaneously ^​1​.

Systemic chemotherapy gets into the bloodstream to reach cancer cells in the body. Ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a swallowed pill. However, chemotherapy for Ewing sarcoma is usually injected into a vein or muscle; it is rarely given by mouth.

When possible, treatment for Ewing sarcoma begins with chemotherapy. After this first chemotherapy is finished, the doctor can use localized surgery or radiation therapy followed by more chemotherapy to eliminate any remaining cancer cells. 

A chemotherapy regimen typically consists of a specific number of cycles over a set period. A patient can receive one drug at a time or combinations of different drugs given simultaneously. Children and young adults with Ewing sarcoma should receive doxorubicin (Adriamycin), cyclophosphamide (Cytoxan, Neosar), etoposide (Toposar, VePesid), ifosfamide (Ifex), or vincristine (Oncovin, Vincasar PFS) ^​2​. The standard schedule for Ewing sarcoma that has not spread to other body parts is chemotherapy every two weeks. Patients with metastatic Ewing sarcoma can also be treated with the above medications and dactinomycin (Cosmegen).

The side effects of chemotherapy depend on the patient and the dose used. Still, they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhoea. These side effects usually go after treatment is completed.

Children and young adults receiving chemotherapy for Ewing sarcoma may be at risk for developing neutropenia, which is an abnormally low level of a type of white blood cell called neutrophils. White blood cells help the body fight infection. The doctor can give the patient medications to increase their white blood cell counts. These medications are called white blood cell growth factors, also called colony-stimulating factors (CSFs). Treating neutropenia is an essential part of the overall treatment plan.

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. When possible, surgical removal of the tumor should happen after chemotherapy. Surgery may also be needed to remove any remaining cancer cells after chemotherapy or radiation therapy ^​3​. An orthopaedic oncologist is usually the doctor who will perform the surgery.

Often, a tumor can be removed without causing disability. However, if the tumor occurs in an arm or leg, surgery to remove much of the bone may affect the limb’s ability to function. Bone grafts from other body parts may help reconstruct the limb, and a prosthesis made of metal or plastic bones or joints can replace lost tissue. Physical therapy after surgery can help children and young adults learn to use the limb again. 

Radiation Therapy

Radiation therapy uses high-energy X-rays to kill cancer cells ^​4​. A radiation therapy schedule consists typically of a specific number of treatments offered over a set period.

Radiation therapy can be combined with chemotherapy during treatment.

The most common radiation treatment type is external-beam radiation therapy, given from a machine outside the body.

When radiation treatment is given inside the body directly, it is internal radiation therapy or brachytherapy. 

Side effects from radiation therapy may include upset stomach, fatigue, mild skin reactions, and loose bowel movements. Most side effects go away soon after treatment is finished. In the long term, radiation therapy can also interfere with normal bone growth and increase the risk of secondary cancer. 

Stem cell Transplantation/Bone marrow Transplantation

A stem cell transplant is a medical process in which bone marrow that contains the cancer is replaced by highly specialized cells. These cells, known as hematopoietic stem cells, develop into the healthy bone marrow. Hematopoietic stem cells are the blood-forming cells found in the bloodstream and the bone marrow. These stem cells form all of the healthy cells in the blood. Presently, this procedure is more commonly called a stem cell transplant than a bone marrow transplant because it is the stem cells in the blood typically being transplanted, not the actual bone marrow tissue.

Before suggesting transplantation, doctors talk to the patient and family members regarding the risks of this treatment. They will also consider several other factors, like age and general health, cancer type, and previous treatment results.

There are two types of stem cell transplantation depending on the source of the replacement blood stem cells – autologous (AUTO) and allogeneic (ALLO) ^​5​. AUTO uses the patient’s stem cells, while ALLO uses donated stem cells. In both types, the target is to destroy all cancer cells in the blood, marrow, and other body parts using high doses of chemotherapy or radiation therapy and then allow replacement blood stem cells to create healthy bone marrow.

Side effects depend on the child’s general health, the type of transplant, and other factors.

Palliative Care 

Cancer and its treatment have side effects that can be mental, physical or financial and managing the effects are palliative or supportive care.

Palliative care includes medication, nutritional changes, emotional and spiritual support and other relaxation therapies. 

Palliative care focuses on alleviating how you feel during treatment by managing symptoms and supporting patients and their families with other non-medical needs. Regardless of type and stage of Cancer age, any person may receive this type of care.

Remission and chance of recurrence

When cancer can’t be detected in the body and there are no symptoms, this is known as remission. This may be called having ‘no evidence of disease’.

A remission can be temporary or permanent. Many people worry about the recurrence of cancer.

The doctor performs another round of tests to know the extent of the recurrence.

Mainly the treatment plan includes the treatments explained above, like surgery, chemotherapy, radiation therapy, and targeted therapy.

 Recurrence is most common within the first two years after Ewing sarcoma treatment has finished. But late recurrences that develop up to 5 years after treatment are more common with Ewing sarcoma than with other types of cancers in this age group.

If there is a recurrence, a new cycle of testing starts again to know as much as possible about the recurrence. After this testing is done, the doctor will talk about the treatment options with you. The next round of treatment will depend on where and when cancer recurred and how it was first treated. The doctor may suggest chemotherapy, including cyclophosphamide (available as a generic drug) and temozolomide (Temodar), irinotecan (Camptosar), and topotecan (Hycamtin, Brakiva); radiation therapy; or surgery to remove new tumours. Bone marrow/stem cell transplantation can also be recommended.

If treatment doesn’t work

If cancer can’t be treated or controlled, it leads to advanced or terminal cancer. It is vital to have straightforward conversations with your health care team to express your feelings, preferences, and concerns. The health care team has unique skills, knowledge, and experience to assist patients and their families. Ensuring that a person is physically comfortable, free from pain, and emotionally supported is extremely important.

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