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Chemotherapy Regimens for Recurrent Ewing Sarcoma

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High-grade cancer that grows in bone or the body's soft tissues is called Ewing's sarcoma. Continue reading to know more.

Medically reviewed by

Dr. Abdul Aziz Khan

Published At September 11, 2023
Reviewed AtSeptember 11, 2023

Introduction:

Sarcoma is a specific kind of cancer in the body's soft tissues, such as cartilage, fat, muscle, blood vessels, fibrous tissue, or other connective or supportive tissue in the bone. Based on how cancer develops, there are various types of sarcoma. Chemotherapy, also known as chemo, is a common treatment for cancer, but it is also used to treat other illnesses. Salvage chemotherapy is considered when the disease reoccurs and when initial chemotherapy fails to give complete results.

What Is Ewing's Sarcoma?

Cancer that forms in the soft tissue or bone is known as Ewing sarcoma. Ewing’s sarcomas are also known as a peripheral primitive neuroectodermal tumor. It is a rare type of cancer that mostly occurs in children or young adults.

What Are the Causes of Recurrent Ewing Sarcoma?

Certain cells modify their DNA (genetic material), causing Ewing sarcoma. The DNA of cells directs their actions. When a cell has Ewing sarcoma, its DNA advises healthy cells to disobey earlier signals which would have killed the cell. With their new orders, the healthy cells continue to grow and eventually form irregular cells. Ewing sarcoma is brought on by these abnormal cells destroying healthy cells. When an Ewing sarcoma fails to respond to treatment or occurs again is called recurrent Ewing's sarcoma.

What Are the Symptoms of Ewing’s Sarcoma?

  • Pain and swelling in the site of cancer cells.

  • Fever.

  • Lump in the chest, legs, arms, or pelvis.

  • Easy breaking of bone is observed.

How Can Ewing's Sarcoma Be Diagnosed?

Ewing's sarcoma can be diagnosed in the following ways -

  • X-Ray- A diagnostic method that uses electromagnetic radiation to visualize the internal organs for the presence of sarcoma.

  • MRI (Magnetic Resonance Imaging) - It is an imaging method that uses magnetic fields and computer-generated radio waves to visualize the sarcoma present inside the body. It depicts the number of sarcomas and their size and shape.

  • CT Scan (Computed Tomography) - A medical imaging technique that utilizes X-rays and computer technology to identify bone destruction associated with Ewing's sarcoma.

  • PET Scan (Positron Emission Tomography) - It is a diagnostic method that reveals the metabolic function of tissues.

  • Bone Scan - It is a method that utilizes nuclear imaging technology to diagnose the condition of the bone.

Laboratory investigations include the following.

  • Bone Marrow Aspiration - A needle is used to extract a little sample of bone marrow for analysis.

  • Biopsy - A part of the affected tissue is removed and examined.

  • Complete Blood Count - A blood test that gives information about the quality and quantity of different types of cells in the blood.

How Often Does Ewing Sarcoma Recurrence Occur?

Most of the time, the first recurrence can be seen in the lungs and bones. This pattern of recurrence at a place distant from the first location is common. As opposed to systemic relapse, isolated local recurrence is more frequent in people who initially had localized tumors.

Interestingly, in one large case series, roughly half of recurrent patients were identified by routine surveillance imaging. In contrast, the other half were symptomatic when recurrence was discovered, with new pain or swelling being the most common complaint.

What Are the Treatment Options for Ewing Sarcoma?

The various treatment options are

1. Radiotherapy - The process of killing cancer cells and reducing tumors by exposing them to high-energy radiation from sources such as x-rays, gamma rays, neutrons, and protons

2. Autologous Stem Cell Transplant - A procedure in which the patient's healthy stem cells (blood-forming cells) are removed from their blood or bone marrow before therapy, preserved, and then given back to them after treatment. An autologous stem cell transplant replaces a patient's stem cells that have been destroyed by radiation therapy or aggressive chemotherapy.

3. Chemotherapy - Chemotherapy is a system-wide treatment because the drugs it includes circulate throughout the body and can eradicate cancer cells that have either started to spread or have already reached areas outside the initial tumor. For all this reason, it is unique from therapies like surgery and radiation.

  • Chemotherapy Regimens for Recurrent Ewing Sarcoma: Combination chemotherapy is usually used to treat patients with recurrent Ewing sarcoma. In opposition to initial treatment methods, chemotherapeutic techniques or the management of recurrent Ewing's sarcoma disease typically utilize various drugs and treatments with various effects. However, people may respond to medications in the initial treatment method if they have a long period without developing a condition after stopping the first treatment. No approved chemotherapy treatments are now available for people with recurrent illnesses.

  • Conventional Dose Chemotherapy: Patients with recurrent Ewing's sarcoma receive certain medications like Cisplatin and Etoposide. The researchers viewed this as an effective symptom-relief strategy. Combination chemotherapy based on Cisplatin is also used. In both cases, there was a very minute percentage of complete response, whereas a major percentage of partial response is noted.

  • High-Dose Chemotherapy Autologous Stem Cell Transplant: This method is now a standard approach for recurrent Ewing's sarcoma and is highly used. High-dose chemotherapy with autologous stem cell transplant may increase survival in individuals with recurrent Ewing's sarcoma that responds to chemotherapy. The overall survival rate for patients who underwent stem cell transplantation was 75 % compared to 20 % of patients who did not. Patients who responded to treatment had a higher survival rate. Patients who went longer than two years without experiencing a recurrence also fared better in terms of survival. It confirms that patients with recurrent or refractory Ewing's sarcoma treated with high-dose chemotherapy can achieve long-term survival. Studies are being carried out to identify the most efficient treatment.

4. Surgery - Surgery is mostly done after chemotherapy and involves removing cancer. However, if the recurrence is in the lungs, cancer should be checked to see if it can be removed from surgery.

Conclusion:

In the beginning, salvage chemotherapy may help many people by decreasing their symptoms and delaying the course of their condition, but those reliable treatments continue to be complicated. To successfully treat Ewing's sarcoma, patients must get care from a multi-disciplinary team that includes their primary care physician, an orthopedic surgeon with experience in treating bone tumors, a pathologist, a radiation oncologist, a pediatric oncologist, a rehabilitation specialist, a pediatric nurse specialistic, social workers and others. Specialty cancer clinics that treat patients with Ewing's sarcoma are the best places to find experienced staff.

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Dr. Abdul Aziz Khan
Dr. Abdul Aziz Khan

Medical oncology

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