Ewing Sarcoma: Understanding the Rare Bone Cancer in Children and Young Adults

Key Takeaways

  • This aggressive bone cancer strikes predominantly between ages 5-25, with 87% of cases developing in the femur, pelvis, ribs, or humerus rather than soft tissues.
  • Characteristic pain worsens at night and during physical activity, often accompanied by localized swelling and unexplained fractures at the tumor site.
  • Unlike many cancers, no hereditary, dietary, or environmental risk factors have been identified—cases appear to result from random DNA mutations occurring after birth.
  • Standard treatment follows a three-phase protocol: initial chemotherapy for 10-12 weeks to shrink tumors, followed by local control through surgery or radiation, then additional chemotherapy.
  • Survival rates drop dramatically with metastasis—from 82% five-year survival for localized disease to just 39-71% when cancer has spread to lungs or other bones.

Ewing sarcoma is a rare type of bone cancer that primarily affects children and young adults. This condition occurs when cancerous tumors grow in the bones or the soft tissues surrounding the bones, such as cartilage. Although Ewing's sarcoma is uncommon, it is essential to understand the types, causes, symptoms, diagnosis, and treatment options for this condition.

Types of Ewing Sarcoma

There are four main types of Ewing sarcoma, depending on where the tumor forms:

  • Bone tumor: The most common type, accounting for 87% of cases, usually affecting the thigh bones, pelvis, ribs, or upper arms.

  • Soft tissue tumor: This type grows in the soft tissues around the bones, such as cartilage.

  • Peripheral primitive neuroectodermal tumor (PNET): Found in the nerves, this type can grow in various parts of the body.

  • Askin tumor: A type of PNET tumor that develops in the chest wall.

All types of Ewing sarcoma have the potential to spread to other parts of the body, including the bones, bone marrow, and lungs.

Ewing Sarcoma: Understanding the Rare Bone Cancer in Children and Young Adults

Causes of Ewing Sarcoma

The exact cause of Ewing sarcoma remains unknown. Researchers believe that changes in a cell's DNA occurring after birth are likely responsible for the development of this cancer. However, it is not linked to family history, diet, lifestyle, or environmental factors.

Symptoms of Ewing Sarcoma

Common symptoms of Ewing sarcoma include:

  • Pain, swelling, or stiffness in the area of the tumor

  • A lump near the skin that feels warm and soft to the touch

  • Persistent low-grade fever

  • Limping due to leg pain

  • Bone pain that worsens with exercise or at night

  • Unexplained bone fractures

  • Unintentional weight loss

  • Fatigue without a known reason

Diagnosis and Tests for Ewing Sarcoma

To diagnose Ewing sarcoma and determine if the tumor has spread, doctors may perform several tests, including:

  • Physical exam

  • X-rays

  • Bone scan

  • Magnetic resonance imaging (MRI)

  • Computed tomography scan (CT scan)

  • Positron emission tomography scan (PET scan)

  • Biopsy

  • Bone marrow aspiration and biopsy

Treatments for Ewing Sarcoma

Treatment options for Ewing sarcoma depend on factors such as tumor size, location, spread, the child's overall health, and family preferences. The main treatment options include:

  • Chemotherapy: Usually the first step, involving medication to shrink the tumor.

  • Surgery: Typically follows chemotherapy, aiming to remove the tumor and prevent its spread.

  • Radiation therapy: Uses X-rays and other types of radiation to kill cancer cells.

Ewing Sarcoma Survival Rate

The survival rate for Ewing sarcoma depends on whether the cancer has spread. According to the American Cancer Society, if the cancer hasn't spread, the 5-year survival rate is 82%. If the cancer has spread, the survival rate ranges from 39% to 71%, depending on the extent of the spread. It is important to note that these are estimates, and survival rates continue to improve with advancements in treatment.

For more information on Ewing sarcoma, visit the National Cancer Institute and the St. Jude Children's Research Hospital websites.

The Bottom Line

Early detection of this rare but treatable cancer is crucial, as localized cases have excellent survival rates with aggressive multimodal therapy. Persistent bone pain that worsens at night, especially in teens and young adults, warrants immediate evaluation. If you're experiencing unexplained bone pain or concerning symptoms, Doctronic can help connect you with appropriate specialists quickly.

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