Ewing’s sarcoma is a primary bone cancer; it is one of a group of cancers known collectively as the Ewing’s sarcoma family of tumors (ESFT). Ewing sarcoma is a type of tumor that forms in the bone or soft tissue. It is considered a rare type of cancer that is often overlooked and receives minimal recognition and research funding. Each year about 225 children and teenagers in the US are diagnosed, accounting for 30% of all bone cancers that occur in children. Although, Ewing’s sarcoma is considered a pediatric cancer about 20 cases per year are found in adults. The most commonly affected areas include the pelvis, thigh, lower leg, upper arm, and chest wall.

The mutation that causes Ewing’s sarcoma involves two genes, the EWSR1 gene on chromosome 22 and FLI1 gene on chromosome 11. A rearrangement (translocation) of genetic material between chromosome 22 and 11, t(11;22), fuses part of the EWSR1 gene with part of the FLI1 gene, creating the EWSR1/FLI1 fusion gene. This fusion gene occurs in approximately 85% of Ewing’s sarcomas. The EWSR1/FLI1 protein turns a variety of genes on and off abnormally, leading to uncontrolled growth promoting tumor development.

ES in not an inherited condition. The following factors may increase your chance of developing Ewing’s sarcoma:

•   History of another congenital disease

• Previous exposure to treatment using radiotherapy

• Race: People who are of African American or Asian decent are at a significantly lower risk of developing Ewing’s sarcoma than Caucasians.

• Age: the tumor is more common among teenagers; about 25% of cases occur before age 10, while 75% affected patients are between the age of 10-20 years old.

• Gender: In post adolescent onset, the cancer is more common in males.

• Episodes of excruciating pain and/or swelling in the arm, leg, pelvis, back, chest wall, or thigh

• Bone pain not associated with growing pains

• Pain that intensifies at night

• Pain/tenderness in skull

• Pain that increases with weight bearing activities

• Swelling that may or may not feel warm

• Limited range of motion of a joint

• Difficulty breathing; tumor metastasizes early to the lungs

• Fever; low-grade

• Bone fracture not caused by injury

• Lump formation that does not subside over time; possible cyst in bone

• Weight loss; loss of appetite

• Fatigue, low energy levels; excessive sleepiness

Often times, the symptoms of Ewing’s sarcoma are mistaken for growing pains or the effects of strenuous exercise. These symptoms include persistent pain and/or swelling in the bone, or in the tissue surrounding the bone. The formation of a lump indicates tumor growth that may feel warm and sensitive to the touch. As the tumor grows in the bone it can cause it to become weak resulting in possible fractures. Difficulty breathing if the tumor is in the chest wall and persistent fever are some common symptoms of ES.

Once your symptoms and medical history are reviewed, your doctor will perform a physical exam. You will then be treated by a team of doctors consisting of an oncologist (doctor who specializes in cancer), orthopedic surgeon (doctor who operates on bones), and a radiation oncologist (doctor who uses radiation in the treatment of cancer.) For proper diagnosis, imaging tests such as an X-ray, bone scan, CT scan, PET scan, and MRI may be performed. Such tests can suggest that cancer is present, but a biopsy and a bone marrow aspiration can make a definitive diagnosis.

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In treating any type of cancer, to achieve a favorable prognosis, early detection is critical. However, for Ewing’s sarcoma there is a considerable delay in diagnosing patients. In the last 20 years, studies have shown little improvement in this area. Raising awareness and becoming educated on early signs and symptoms will potentially reduce delays in diagnosis.

More than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments with new and improved methods of diagnosing, treating, and preventing different diseases. Prior to treatment, the staging of Ewing’s sarcoma is determined; classifying the tumor as localized or metastatic (spread of cancer to other areas in the body.) The most common sites include the lungs, other bones, and bone marrow. Treatment for ES usually involves a combination of chemotherapy, localized surgery, and radiation therapy. Radiation therapy is often introduced if the surgery did not successfully remove all of the tumor cells, or in the event where surgery is not an option. Relapse rates have shown to be high in patients using localized therapy alone (80-90%); therefore, administration of chemotherapy resumes after surgery or radiation. For metastatic cancer high dose chemotherapy is often recommended, but it can also destroy the bone marrow. In this case, Stem Cell Transplantation may be introduced, where the goal is to allow replacement blood stem cells to create healthy bone marrow. The prognosis for Ewing’s sarcoma: five-year survival for localized disease is 70-80% when treated with chemotherapy, for metastatic cancer it drops dramatically to 15-30%.

The latest treatments also include the use of:​

• Combination chemotherapy-cancer cells are attacked by multiple drugs with synergy; medicine’s best attempt to prevent mutation and resistance

• Bilateral lung radiation for metastatic Ewing’s sarcoma, radiation is administered after chemotherapy

• Insulin-like growth factor receptor-1 (IGF-1R) antibodies is an important protein for ES, blocking its activity may lead to new ways in treating ES.

• Palliative care- clinical trials are experimenting on ways to reduce symptoms and side effects in order to improve patient’s quality of life.

• Immunotherapy-using the body’s immune system to fight cancer cells

  • Adoptive T Cell Therapy- T cells are extracted from the patient, genetically modified and returned to the patient with the hopes of boosting the immune system to fight cancer cells

  • Therapeutic Vaccines- Vaccines are designed to induce an immune response against tumor-specific or tumor-associated antigens

  • Checkpoint Inhibitors/ Immune Modulators- Treatment works by targeting molecules involved in the regulation of the immune system

  • Oncolytic Virus Therapy- Virus therapy uses a modified virus that works by infecting and killing tumor cells, and stimulating an immune response

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For more information on clinical trials visit: ClinicalTrials.gov

(Courtesy of The National Library of Medicine)

Filling the funding gap - One in every 330 Americans will develop cancer before the age of 30, yet pediatric cancer does not receive nearly as much funding as adult research cancer projects. Only 4% of the federal budget is allocated to pediatric cancer research. Pharmaceutical companies fund virtually nothing towards pediatric cancer research, because childhood cancer drugs are deemed unprofitable.

If you would like to learn more about paying for cancer treatments, there is additional information in this article published by The Simple Dollar.

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