Primitive neuroectodermal tumor Essay Sample
Primitive neuroectodermal tumor, or Ewing’s sarcoma, is an atypical disease in which cancer cells are found in bone or within the soft tissue. Ewing’s sarcoma is most common in the pelvis, femur, humerus, and the ribs (Strauss, 2005). This disease occurs most regularly in male teenagers. Ewing’s sarcoma is the result of a translocation between chromosomes 11 and 22, which fuses the EWS gene of chromosome 22 to the FL11 gene of the chromosome 11 (Strauss, 2005).
Ewing’s sarcoma is most common in males and most likely presents in childhood or early adulthood – a peak between 10 to 20 years of age (Strauss, 2005). This sarcoma is exclusively in caucasians. It has the ability to occur or show up anywhere in the body, but primarily in the proximal long tubular bones (i.e. femur, tibia, and humerus) and pelvis region (Strauss, 2005). The clinical findings include pain and swelling; and thirty percent are exaggeratedly metastatic at presentation (Staruss, 2005).
In the United States, the incidence of Ewing’s sarcoma in individuals younger than three years of age is .3 cases per one million, and adolescent cases 15-19 years of age significantly increases to 4.6 cases per one million (Strauss, 2005). Annual cases internationally average less than two cases per one million individuals. Within the United Kingdom an average of six cases are diagnosed and reported annually, chiefly males in the early stages of puberty (Strauss, 2005).
The treatment of Ewing’s sarcoma is a multi-drug chemotherapy with a local disease control, such as surgery or radiation due to the occult metastatic progression. A complete biopsy may also be performed if the malignity is confirmed (Strauss, 2005). The length of treatment can vary. Patients can undergo chemotherapy for six to twelve months and radiation for five to six weeks (Strauss, 2005).
In concluding, Ewing’s sarcoma is extremely rare – .3 to 4.6 cases per one million – and promotes cases that are more apparent in males with a ratio near 1.5:1. It is a prejudicial disease and is seldom outside cauasians (Strauss, 2005). Treatment consists of a multi-drug chemotherapy and a localized treatment of surgery and/or radiation. Survival is promising if it is caught within the localized stages with a rate of sixty-five to seventy percent. However, if metastasized survival drops to twenty-five to thirty percent (Strauss, 2005).
Strauss, Ludwig G. (2005). Ewing Sarcoma. 10 June 2005.