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What is Ewing’s sarcoma? It is a rare bone tumor that mainly affects children, adolescents and young adults: the average age at diagnosis is around 15 years. The family of Ewing’s sarcomas includes forms originating from bone (about 85%) and those originating from soft tissue (15%). As explained by the ISS on its ISSalute portal, Ewing’s sarcoma is the second most common bone cancer in children and adolescents, after osteosarcoma and represents 2% of all pediatric cancers.
Incidence: males are more affected
deepening
Lorenzo Bastelli, a 14-year-old suffering from Ewing’s sarcoma, has died
Generally, boys are more affected than girls, with a ratio of 1.6 to 1.0, although Ewing’s sarcoma occurs equally in boys and girls between the ages of 0 and 14 years.
The Ewing sarcoma family includes Ewing’s sarcoma originating in bone, extraosseous Ewing’s sarcoma originating in soft tissue, Askin’s tumor originating in the chest wall, and Primary Neuroectodermal Tumor (PNET).
Symptoms
The symptoms of Ewing’s sarcoma vary from person to person and according to the size and location affected. It usually manifests as intermittent pain in the affected area (bone or near the bone) especially during the night,
mass or swelling around the affected bone or joint, limitation of movement in the joints e
spontaneous fracture of the affected bone. Other less common ailments may be fever,
fatigue, tiredness, weight loss and difficulty breathing. As underlined by the Higher Institute of Health, “the suspicion of the disease is almost never immediate both due to the rarity and because the non-specific disorders can resemble those present in other conditions or diseases”, such as trauma from sports,
tendonitis, bone infection or
growing pains in children.
Cause
At the moment, the ISS specifies, the possible causes, nor the risk factors of Ewing’s sarcoma are not known. There are also no known environmental risk factors or hereditary factors capable of increasing the risk of the disease.
Diagnosis
The determination of Ewing’s sarcoma requires a biopsy and analysis of the tissue removed. Generally, patients go to their general practitioner or pediatrician when the symptoms appear. “However, the non-specificity of the disorders and the rarity of the disease generally tend to delay their discovery, because the doctor does not suspect it. The disorders are often traced back to “growing pains” or trauma from sporting activity”, specifies the ISS. The diagnostic workup includes blood tests, x-rays and magnetic resonance imaging to confirm the suspicion of cancer. We then proceed with the biopsy, i.e. the taking of a fragment of tissue which will then be analyzed in the laboratory, with consequent diagnosis on the tissue taken.
Therapy
The therapy against this neoplasm requires a multidisciplinary approach, through a team of specialists, in reference centers for sarcomas. Treatments are usually “multimodal,” meaning they include a combination of chemotherapy and surgery and/or radiation therapy. “In localized disease, chemotherapy is usually used both before surgery, to reduce the tumor mass to be removed and target any micrometastases, and after surgery, to eliminate cancer cells that may have spread throughout the body,” explains l ‘Iss, underlining that “the combinations of drugs, the number of cycles and the interval between administrations are mainly related to the stage of the disease but also to the age of the patients”. For patients whose Ewing sarcoma recurs within 2 years of diagnosis, there are currently no specific treatments available. Surgery that completely removes the tumor remains the main treatment locally. “The improvement of surgical techniques today allows, especially in the limbs, conservative and reconstructive surgery in almost 90% of cases, avoiding demolition operations (amputations). However, it is not always possible to remove the tumor en bloc”, specified the Higher Institute of Health.