Ewing Sarcoma is a highly aggressive, malignant bone tumor or soft-tissue tumor occurring predominantly in the bones of pelvis, femur, tibia and ribs or in the soft tissues of thoracic wall, gluteal muscle, pleural cavities and cervical muscles. It mainly affects children, adolescents and young adults.
The WHO classification of sarcomas (2013) uniformly defined ‘Ewing sarcoma’ by characterized pathognomonic FET–ETS gene fusions. The common factors of Ewing Sarcoma includes swelling and soreness around the tumor area, low fever, bone pain, especially pain that worsens during exercise or at night, limping, which is caused by a tumor on a leg bone and others. According to Cancer UK, Ewing tumors of bone account for 37% of the bone tumors in children.
Up to 25 children per year are diagnosed in the UK. As per the study of Jane et. al., Ewing Sarcoma distribution is relatively even across the various sites: head (17.7%), upper extremity (17.7%), lower extremity (11.8%), pelvis (17.7%), chest (14.7%), abdomen (11.8%), and spine (8.8%).
The diagnosis of Ewing Sarcoma includes physical examination and Magnetic Resonance Imaging (MRI), CT Scan, Positron Emission tomography scan (PET), Bone Scan, Bone marrow aspiration and biopsy, X-ray, Complete blood count (CBC), and other studies. The multimodal treatment includes surgical resection and/or local radiotherapy as well as intensive multi-agent chemotherapy.
Ewing Sarcoma is very common in teenagers and young adults. According to SEER registry, it is predominantly observed in populations of European descent with an estimated incidence of ~1.5 cases per million children and adolescents and young adults, for which a genetic explanation has been proposed. It is rare in Japanese people, according to Japanese Orthopedic Association (JOA) registry, only 30–40 patients develop Ewing Sarcoma annually.
The successful treatment of patients with Ewing sarcoma requires systemic chemotherapy in conjunction with surgery and/or radiation therapy for local tumor control. In general, patients receive chemotherapy before instituting local-control measures. In patients who undergo surgery, surgical margins and histologic response are considered in planning postoperative therapy. Patients with metastatic disease often have a good initial response to preoperative chemotherapy, but in most cases, the disease is only partially controlled or recurs. Patients with lung as the only metastatic site have a better prognosis than do patients with metastases to bone and/or bone marrow. Adequate local control for metastatic sites, particularly bone metastases, may be an important issue.
For more info- Ewing Sarcoma Prevalence
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