What is Ewings Sarcoma

What is Ewings Sarcoma and PNET?

What is Ewings Sarcoma and PNET?

Ewing’s sarcoma is an uncommon cancer characterized by a solid tumor, most frequently observed in adolescents and children, with the majority of cases emerging between the ages of 10 and 20. Traditionally categorized as a bone cancer due to its primary occurrence within bones, it actually originates from a type of primitive nerve cell. Consequently, Ewing’s sarcoma can manifest outside of the skeletal system, affecting the body’s soft tissues.

Primitive neuroectodermal tumor (PNET), also known as peripheral neuroepithelioma, was once classified as a distinct cancer from Ewing’s sarcoma. However, contemporary understanding suggests they represent different manifestations of the same disease. Treatment strategies and responses to therapy are akin for both. In cases where PNET occurs in the chest wall, it’s referred to as Askin’s tumor. PNET can also manifest in the testes, potentially leading to confusion with testicular germ cell cancer. Specialized laboratory analyses are conducted to ascertain the nature of abnormal cells, as treatment protocols for PNET differ from those for typical testicular cancer.

How does Ewing’s sarcoma impact the body

Ewing’s sarcoma typically originates in the arms or legs, though it can also arise in the pelvis, ribs, spine, and occasionally other bones or soft tissues. Upon diagnosis, approximately 23% to 26% of cases have already spread (metastasized) to other sites, commonly the lungs, a second bone, or the bone marrow. Similar to other cancers, Ewing’s sarcoma stems from abnormal cells that proliferate uncontrollably. These cells exhibit an altered genetic makeup inherited from parents (DNA), characterized by a translocation, often involving a rearrangement between chromosome 22 and chromosome 11. However, this genetic anomaly is not inherited but rather develops post-birth. Despite extensive research, the exact cause of Ewing’s sarcoma remains elusive, with no conclusive evidence linking it to radiation exposure, chemicals, or other environmental factors.

What are the available choices for treatment

Typically, Ewing’s sarcoma is addressed through a combination of chemotherapy, radiation therapy, and/or surgical intervention. Surgery or radiation therapy may be employed to remove or shrink the tumor, aiming to halt its growth or prevent its spread.

Surgical removal of the tumor is often pursued to preserve the affected limb, and reconstructive techniques like bone grafting or the use of prosthetics may be employed to maintain limb functionality. In cases where the cancer has metastasized to the lungs, thoracotomy (open chest surgery) may be necessary.

Radiation therapy is another crucial treatment modality for Ewing’s sarcoma, particularly effective in reducing tumor size and preventing recurrence. The choice of treatment depends on factors such as tumor size and extent of tissue involvement.

Chemotherapy is frequently administered alongside radiation therapy and/or surgery in Ewing’s sarcoma treatment regimens. It may be administered before surgery to facilitate tumor removal or as a standalone treatment to minimize the risk of metastasis or tumor progression. Chemotherapy drugs work to inhibit tumor growth and dissemination, reducing the likelihood of spread to distant sites such as the lungs or bone marrow.