Ewing's sarcoma
Ewing's sarcoma is a rare and aggressive form of bone cancer that primarily affects children and young adults, typically between the ages of five and twenty-five. It most commonly manifests in the long bones (60%), pelvis (18%), and ribs (15%). The exact cause of Ewing's sarcoma remains unknown, though it may be linked to factors such as recurrent trauma, congenital anomalies, and exposure to ionizing radiation. Symptoms usually begin with intermittent pain in the affected area, which can escalate to intense discomfort, accompanied by swelling, weight loss, fever, and mild anemia.
The disease can progress through different phases: localized, regional, and distant metastasis. Treatment is complex and may include surgery, chemotherapy, radiation, and high-dose chemotherapy with stem cell rescue, depending on the tumor's location and stage. While localized Ewing's sarcoma has a long-term survival rate of 50 to 70%, metastatic cases present a significantly lower survival rate of 15 to 30%. Ongoing research into immunotherapy and targeted treatments offers hope for improved outcomes, highlighting the importance of early diagnosis and a multidisciplinary approach to care.
Ewing's sarcoma
ALSO KNOWN AS: Askin tumor, bone cancer, bone neoplasm--Ewing sarcoma, Ewing family of tumors, Ewing sarcoma, extraosseous Ewing sarcoma, primitive neuroectodermal tumors (PNET)
ANATOMY OR SYSTEM AFFECTED: Bones, musculoskeletal system
DEFINITION: A rare bone cancer involving any part of the skeleton but found commonly in the long bones (60 percent), the pelvis (18 percent), and the ribs (15 percent) of children and young adults.
CAUSES: Unknown; possibly related to recurrent trauma, metal implants, congenital anomalies, unrelated tumors, exposure to ionizing radiation
SYMPTOMS: Pain in long bones, vertebra, or pelvis; swelling; neurological disorders; weight loss; fever; mild anemia
DURATION: Long-term
TREATMENTS: Surgery, chemotherapy, radiation, high-dose chemotherapy with stem cell rescue
Causes and Symptoms
The specific cause of Ewing’s sarcoma is unknown, but it may be associated with recurrent trauma, metal implants, congenital anomalies, unrelated tumors, or exposure to ionizing radiation. Approximately 90 percent of patients are between five and twenty-five years of age; rarely are patients younger than five or older than forty.
The initial symptom is pain, discontinuous at first and then intense, in the long bones, vertebra, or pelvis. Swelling may follow. Neurological signs involving the nerve roots or are characteristic of nearly one-half of patients with involvement of the axial skeleton. Weight loss may occur, with remittent fever and mild anemia.
The phases of Ewing’s are based on degree of metastasis: the local phase (a nonmetastatic tumor), the regional phase (lymph node involvement), and the distant phase (involvement of the lungs, bones, and sometimes the central nervous system).
Treatment and Therapy
Patients are of two types, those with localized tumors and those with metastasized tumors. Depending on where the tumor is located, the treatment of Ewing’s sarcoma is complex in all stages of disease and requires a multidisciplinary perspective. It is best treated when diagnosed early. Obtaining a bone is recommended in nearly all cases.
Surgery may be used to remove a tumor, followed by administered to kill any remaining cancer cells. Radiation may be prescribed to kill cancer cells and shrink tumors. Stem cell rescue and transplant to replace stem cells in bone marrow, which are killed by high-dose chemotherapy, is another option. Some patients may benefit from tandem stem cell transplants, or two procedures spaced a few months apart. Clinical trials are exploring other options including immunotherapy and targeted therapy.
Perspective and Prospects
Ewing’s sarcoma is one of the most malignant of all tumors. It may be localized or metastasize to the and other bones. The primary can be controlled by irradiation, but the is poor. Often, amputation is not justifiable. Recent developments in multiagent chemotherapy, however, are encouraging. Long-term survival of patients with Ewing’s sarcoma is 50 to 70 percent or more with localized disease; the rate drops to 15 to 30 percent for metastatic disease. Prognosis is affected by many factors, including the age of the patient and whether the main tumor is on a limb or the trunk.
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