Ewing’s sarcoma is a form of aggressive soft tissue cancer that normally begins to grow in the bones. As few as 200 to 300 people are diagnosed with Ewing’s sarcoma every year, but only a small number of these diagnoses will come in adults.

Ewing’s sarcoma is primarily a childhood cancer, according to the National Institutes of Health. More than 6 out of every 10 diagnoses will affect children and young adults between 10 and 20 years of age. Tragically, the disease can also be extremely fatal. In fact, Ewing’s sarcoma leads to more child deaths than any other form of bone malignancy.

Ewing’s Sarcoma: Aggressive & Often Misdiagnosed

As the name suggests, Ewing’s sarcoma (or Ewing’s tumor) is a type of sarcoma, cancers that develop after cells in connective tissue have mutated abnormally. Unlike carcinomas, which begin in epithelial tissue, sarcomas grow from a variety of soft tissues, like muscles, nerves, blood vessels or bone.

Ewing's Sarcoma Micrograph Small

Ewing’s Sarcoma. Copyright © 2011 Michael Bonert. You are free to share and adapt this image as per the CC BY-SA 3.0.

Ewing’s sarcoma usually begins in bone tissue, the American Cancer Society reports, but very similar tumors can also develop from the soft tissues that surround bone. These malignant growths are called extraosseous (“outside bone”) Ewing tumors. A third type of cancer, known as peripheral primitive neuroectodermal tumors, is often classified among the Ewing family of tumors, as well.

What Causes Ewing Tumors?

Ewing’s sarcoma of the bone, extraosseous Ewing tumors and peripheral primitive neuroectodermal tumors have very similar cell characteristics. Moreover, each cancer is characterized by the same genetic abnormality, in which a gene from one chromosome is accidentally fused with a gene on a different chromosome.

This mutation, which is not believed to be hereditary, is observed in around 85% of Ewing’s sarcoma cases, according to researchers at the University of Utah. Many researchers suggest that this mutation is the cause of Ewing’s sarcoma, but again, the abnormality cannot be passed from parent to child. Instead, the mutation appears to develop in young children and adolescents for no discernible reason, as the experts at Johns Hopkins University point out.

In principle, Ewing’s sarcoma can begin to develop in any bone or soft tissue, but certain bones are far more likely than others:

  • pelvic bone
  • thigh bone (femur)
  • shin bone (tibia)
  • long bone in the arm (humerus)
  • rib
  • collar bone (clavicle)

Like Ewing’s sarcoma itself, sarcomas are extremely rare. Accounting for about 13,000 diagnoses annually, sarcomas represent under 1% of all malignancies diagnosed in the United States.

Early Diagnosis Is Crucial To Survival

As with every type of cancer, diagnosing Ewing’s sarcoma early is crucial to a child’s chances at survival. When appropriate treatment can be administered, the disease can often be cured, according to the University of Chicago Medical Center. In children without metastases, the survival rate for Ewing’s sarcoma lies between 60% and 70% after chemotherapy and surgery.

Unfortunately, many children who are diagnosed with the condition do not benefit from recent advances in medical science. At least 1 in 4 of every Ewing’s sarcoma patients will be diagnosed after the cancer has already spread, usually to the lungs. The remaining 75% of patients, however, are unlikely to have only localized cases, ones confined to the bone or other soft tissue in which they began. In reality, most patients who are diagnosed with “localized” Ewing’s sarcoma actually have micrometastases, the University of New Mexico reports. In these cases, the cancer cells have spread – but are too small for detection with current diagnostic techniques.

The presence of imperceptible micrometastases makes tumor recurrence extremely likely, unless physicians choose an equally aggressive course of treatment. As a result, the majority of people with Ewing’s sarcoma, most of whom are children, will require surgery, systemic chemotherapy and radiation. Children who are diagnosed after their cancer has metastasized face a heart-breakingly poor prognosis, according to St. Jude’s Children’s Research Hospital, surviving in less than 30% of cases.

Why Is Ewing So Easily Missed?

Ewing’s sarcoma is difficult, but not impossible, to diagnose early. As we’ve seen, the cancer’s low rate of early diagnosis can significantly increase the likelihood of an unfavorable prognosis. Getting a diagnosis right the first time is absolutely crucial, but many barriers stand in the way of that goal.

Many physicians have never encountered a pediatric cancer in the clinical setting. Childhood cancers are exceedingly rare, to say nothing of the extreme rarity that distinguishes Ewing’s sarcoma. The National Cancer Institute reports that only around 16,000 cancers are diagnosed in children and adolescents every year. As we mentioned earlier, Ewing’s sarcoma represents only a slight fraction of these cases, around 200 to 300 patients in any given year. For comparison, note that America was home to nearly 92,000 pediatricians in 2011, according to the American Academy of Pediatrics.

How many of those practitioners have ever been faced with a case of Ewing’s sarcoma? Undoubtedly, the answer to that question is very few.

“Non-Specific” Symptoms

To complicate matters, the symptoms caused by Ewing’s sarcoma are “non-specific” and can be related to numerous benign conditions, all of which are far more likely than a childhood cancer. While the disease’s primary sign is extreme bone pain, most young children will experience bone pain at some point in their lives, often due to an accidental injury. As pediatric oncologist Amy Feldman MD writes, “there are many common problems that can result in limb pain in a child,” from sports injuries to infections.

The additional symptoms of Ewing’s sarcoma can also be mistaken for less-serious medical conditions:

  • swelling and / or tenderness at tumor site
  • low-level intermittent fevers (caused by decreased immune system response)
  • unexplained broken bones (bones weakened by a tumor can break even after minor traumatic injuries)
  • weight loss
  • fatigue

It should be clear that Ewing’s sarcoma should never be diagnosed on the basis of symptoms alone. Nor should these troubling warning signs be dismissed out-of-hand as the result of common childhood ailments. Instead, careful pediatricians use a battery of diagnostic techniques to accurately recognize cases of Ewing’s sarcoma.

Diagnosing Ewing’s Sarcoma

In order to differentiate Ewing’s sarcoma from other medical conditions that affect the bones, doctors have a wide array of imaging and laboratory tests at their disposal.

Magnetic resonance imaging (MRI) and computed tomography (CT) can both provide physicians with valuable insight, creating images that may display tumors as large masses of soft tissue, according to the University of Pennsylvania. X-ray presents a more detailed picture, displaying the effect that Ewing’s sarcoma can have as it eats into bone tissue, a process that can result in a characteristic “onion skin” appearance. In addition, bone scans using small amounts of radioactive dye can be conducted to gauge the extent of a tumor and any resulting bone degradation.

As with every type of malignancy, biopsy remains the gold standard technique to diagnose Ewing’s sarcoma. In a biopsy, doctors remove a small amount of tissue from the suspect mass, either using a need or through a surgical incision. Pathologists then inspect the sample’s cell structure under a microscope, examining the tissue closely for signs of disease. Ewing’s sarcoma has been classified as a “small-blue-round-cell tumor,” because it appears as a collection of small round cells that become blue when exposed to a staining dye.

How Are Ewing Tumors Treated?

Without proper treatment, Ewing tumors progress rapidly and become difficult to manage. Thankfully, the cancer is most likely to metastasize first to the lungs, a location that can promise better prognoses than other, more-distant, sites. In any event, physicians normally take an aggressive approach to their treatment of Ewing’s sarcoma, relying on a multi-modal approach, rather than a single therapy.

Most patients will undergo surgery, in which physicians attempt to remove as much of the primary tumor as possible. Since Ewing tumors are distinguished in part by their responsiveness to radiation, a form of radiotherapy may be attempted at this point, too. Radiation is used to pinpoint localized instances of disease that were not removed during surgery. After surgery, most patients will be administered multiple cycles of systemic chemotherapy. Chemotherapy can deal with the undetectable, but often present, micrometastases that many patients with Ewing’s sarcoma have.

Most patients will be treated by an orthopedic oncologist, a specialist in benign and malignant tumors of the bone. Treatment for Ewing tumors is almost always rigorous, and may last up to a year or longer, which can be extremely difficult for pediatric patients.

Can Your Family File A Cancer Misdiagnosis Lawsuit?

Receiving an inaccurate diagnosis, or having a child’s symptoms dismissed out-of-hand, can be devastating. Many patients will lose out on years or months of their lives due to a missed or delayed diagnosis. Others will be subjected to unnecessary treatment methods, which can be excruciating, to due to a mistake in staging. As we’ve seen, Ewing’s sarcoma is particularly likely to be misdiagnosed, in part because even cancer specialists may be unfamiliar with the disease.

Not every case of misdiagnosis, however, is suitable for a medical negligence lawsuit. In a court of law, victims of medical malpractice will need to show that their medical professional violated an accepted standard of care in diagnosis and / or treatment, leading to significant harm. Demonstrating malpractice can be extremely difficult in practice, requiring a wealth of knowledge in the domains of both law and medicine.

Our experienced child injury attorneys can help. To learn more about your family’s legal rights, contact us today for a free consultation.