Ewing Sarcoma: A Rare Cancer Affecting Children and Adolescents

Ewing Sarcoma is a sporadic form of cancer but is quite aggressive, generally affecting children and adolescents. The disease commonly arises in bones or the soft tissues surrounding the bones, including tendons, ligaments, and muscles. Though it may occur at any age, Ewing Sarcoma most often appears between ages 10 and 20. Cancer can start in many different body bones or soft tissues, including the legs or arms, pelvis, chest, spine, or skull, or the soft tissues of the abdomen, arms, neck, or head.

Ewing Sarcoma incidence gives a unique set of challenges to the families and healthcare providers. It is, therefore particularly important to offer timely care or improve patient outcomes through a proper understanding of the disease process, its etiology, symptoms, and modes of treatment.

Causes of Ewing Sarcoma

The exact cause of Ewing Sarcoma is not known to this date. It is, however, known to be caused by mutations/changes in the DNA of cells; these genetic changes are not inherited from the parents. These changes take place on their own and may further result in the uncontrolled multiplication of cells resulting in tumors. Though many studies are going on to know about the genetic mutations, these changes are not inherited and often Ewing Sarcoma in families is a mere coincidence.

Symptoms of Ewing Sarcoma

The signs and symptoms of Ewing Sarcoma will vary depending on the site of the tumor. Most often, children may have the following features:

• Bone pain may periodically come and go but, over time, becomes persistent and increases with any activity
• Swelling, stiffness, or tenderness around the tumour site
• A lump or mass at the site of the tumour
• Fractures of bones that appear to have occurred without significant trauma.

Advanced cases are also manifested by fever, weight loss, and fatigue. These symptoms sometimes look so much like other common childhood conditions that early diagnosis may be difficult. At the same time, persistent bone pain or unexplained symptoms do require urgent medical intervention.

Diagnosis of Ewing Sarcoma

Most of the diagnostic approaches for Ewing Sarcoma in children combine tests and imaging techniques. Some important diagnostic techniques include the following:

• X-rays: These are conducted to detect unusual growth in the bones or lesions.
• Blood tests: These would enlighten one on the patient's general health and also an indication of cancer.
• MRI and CT scans: With these tests, one obtains detailed pictures of the tumour and its extent.
• Bone scans: This helps find out if cancer has reached bones, through the use of a radioactive tracer.
• PET scans: In this technique, the cancerous regions become highlighted in the body, distinguishing cells taking up more radioactive sugar.
• Biopsy: A small amount of tissue is removed from the tumour and analyzed in the lab to confirm cancerous cells are present.

Once diagnosed, physicians determine the tumour's size and location and whether it has spread beyond the original site. This method is called staging and is an important step in cancer treatment.

Staging Ewing Sarcoma

Ewing Sarcoma is either localized or metastatic. Localized tumours are confined to their site of origin or the adjacent tissues, and metastatic Ewing Sarcoma is a tumour that has spread, usually to the lungs, other bones, or bone marrow. Spread to the liver or lymph nodes is less common.

The American Joint Committee on Cancer (AJCC) staging system provides a further categorization of the disease based on:

• T = size/location of the tumour
• N = size of lymph node involvement
• M = metastasis to other organs
• G = grade or appearance of the tumour under the microscope.

Stages: The cancer is given a stage from I to IV based on these factors. Higher stages indicate more extensive disease and dictate treatment options and prognosis.

Treatment Options for Ewing Sarcoma

Multimodal treatment generally consists of surgery, chemotherapy, and radiation therapy in combination. The goal of the treatment is always the complete eradication of tumour tissue together with the preservation of the maximum amount of the affected area, especially when it comes to limbs. Some of the common modes of treatment include the following:

• Surgery: When possible, surgery is performed to remove the tumour along with some normal tissue around it. In limb-sparing surgery, doctors try to save the limb and thus avoid amputation.

• Chemotherapy: This treatment uses strong drugs to kill cancer cells. For most patients, chemotherapy is given before surgery to shrink the tumour and thus make it easier to remove. Chemotherapy after surgery helps to kill the remaining cancer cells.

• Radiation Therapy: It involves using high-energy rays to kill the tumour cells. It is usually applied in cases where surgery cannot remove all the tumours or in instances where surgery is not possible.

• High-Dose Chemotherapy with Stem Cell Transplantation: In cases where the chances of recurrence are very high, a patient may undergo a stem cell transplant. A stem cell transplant involves the use of a high dosage of chemotherapy that is followed by the transplantation of stem cells to revive the bone marrow.

• Clinical Trials: Participation in clinical trials helps the patients get new treatments for the cancer, which may be more helpful and assist the patients in achieving better survival rates with fewer side effects.

Prognosis and Long-Term Outlook

The prognosis for Ewing Sarcoma depends on the size, location, and extent of the disease. Early diagnosis and timely treatment surely make a great difference in the possibility of curing the disease. However, if the cancer has spread to other parts of the body, the chances of a cure are slim. Advancement in research brings lots of hope, and new therapies are emerging with the aim of not only increasing survival rates but also improving the quality of life of the patients.

However, survivors of Ewing Sarcoma can develop complications from the disease process itself or the treatment involved. These include:

• Physical problems, including defects of a limb and dysfunction of a limb.
• Infection, haemorrhage, or graft failure after surgical intervention
• Chemotherapy complications: nausea and vomiting, alopecia, fatigue
• Radiation complications include damage to organs, issues with the growing individual, risk of secondary malignancies
• Emotional/psychological scarring from dealing with the trauma of cancer treatment.

Many children need ongoing treatment, such as physical and occupational therapy, to regain muscle strength and mobility. Counselling and other psychosocial services and support also can help patients cope with the emotional aspects of the disease.

Living with Ewing Sarcoma

Children with diagnoses of Ewing Sarcoma deserve psychological support, as the burden of treatment and rehabilitation falls heavily on them. It is, therefore, necessary to follow up in the long term with periodic radiological tests and follow-up visits with your provider to catch early signs of recurrence or late treatment effects.

Parents and caregivers should ensure that the child receives adequate nutrition, rest, and emotional support during this torrid period. Encourage the balance of rest and physical activities that will help improve general well-being. Be prepared for any side effects or complications: be well-equipped with when to call healthcare providers.

Conclusion

Although extremely rare, Ewing Sarcoma creates devastating difficulties for any young sufferer and their family. With the advances in diagnostic techniques and modalities of treatments available, early intervention now provides the best hope for successful treatment of the disease. Comprehensive care, with further advances in medicine, offers real hope for even higher survival rates and lower long-term complications, hence a brighter future for children diagnosed with this terrible disease.