Ewing’s sarcoma is a highly aggressive form of cancer, characterized by tumors that grow in the bones or the soft tissues that surround bones. While a small minority of adults will be diagnosed with the disease, Ewing’s sarcoma is generally recognized as a childhood cancer, presenting between the ages of 10 and 20 in up to 65% of cases. It is also tragically lethal, causing more child deaths than any other type of bone cancer.
With current medical techniques, Ewing’s sarcoma can be cured in the majority of patients. Before chemotherapy was developed, on the other hand, the five-year survival rate for Ewing’s sarcoma was heartbreakingly low. As many as 80% of children with the disease would die within five years. Today, the outlook is far more positive. When treated prior to metastasis, the prognosis for Ewing’s sarcoma suggests a five-year survival rate of around 70%.
Many children are diagnosed with Ewing’s sarcoma too late. In around 25% of patients, doctors find obvious metastasis on initial imaging tests. A much higher percentage have micrometastases, small regions of spreading cancer that are difficult to identify using modern imaging techniques.
Our statistics, however, may not tell the whole story. As its name suggests, Ewing’s sarcoma is a type of sarcoma, a class of cancers that begin to develop in the body’s connective tissues. While the majority of cancers start in epithelial tissue and are thus classified as carcinomas, sarcoma cancers can develop in tissues as various as muscles, nerves, joints, blood vessels, and bones.
Where Ewing’s sarcoma is concerned, the initial tumors usually develop in:
Once the disease has metastasized, it usually spreads first to a patient’s lungs. In the world of cancer, sarcomas are extremely rare, accounting for only around 13,000 cancer diagnoses every year. Moreover, sarcoma cancers are extremely difficult to differentiate from other types of malignancy. Misdiagnosis is high and our current statistics are likely to capture only a small portion of the sarcomas that actually exist.
Like most cancers, the symptoms of Ewing’s sarcoma can easily be mistaken for other medical conditions. Severe bone pain, for example, is extremely common. But as Dr. Amy Feldman, a pediatric oncologist at the University of Pennsylvania, writes, Ewing’s sarcoma is frequently misdiagnosed, because young children often experience bone pain due to other reasons, including accidental injuries.
At first blush, doctors often mistake the pain caused by Ewing’s sarcoma for more common problems, like:
The disease’s other symptoms can also be difficult to place initially. Swelling and tenderness at the tumor site are explained as a bump or bruise, so common in childhood. Low, intermittent fevers, the result of systemic immune system illness, can be attributed to an infection. Under these circumstances, additional diagnostic tests are necessary.
Physicians have an array of imaging tests at their disposal, from x-rays to magnetic resonance imaging (MRI). Any one of these modalities can be helpful in identifying a tumor and determining the extent of metastasis. For tumors that grow around, but not in, bone tissue, computed axial tomography (CT) scan is preferred, since traditional x-rays have difficulty imaging soft tissues. Ideally, a number of different imaging techniques will be used, since each method has its own strengths and weaknesses. MRI, for example, can capture a tumor’s effect on nearby body structures, like blood vessels, that other modalities may miss.
As in every cancer, a biopsy is the gold standard in the diagnosis of Ewing’s sarcoma. Once a malignancy is suspected, physicians will remove tissue samples from the affected area, either using a large needle or by surgically opening the site. After removal, the sample can be inspected further under a microscope, looking for signs of abnormal cell growth. In many cases, children who may have Ewing’s sarcoma undergo an additional biopsy procedure designed to investigate the bone marrow. In addition, immunohistology tests can confirm the presence of CD99, an antigen produced by Ewing’s sarcoma cancer cells.
The majority of patients with Ewing’s sarcoma will first receive multiple cycles of chemotherapy, intended to reduce the size of their primary tumor. Following chemotherapy, oncologists normally attempt to remove the remaining tumor through surgery. Radiation therapy is an alternative option at this point, depending on the cancer’s location and stage. In some cases, radiation and surgery will be used in tandem. These therapies can be followed by a second round of “adjuvant” chemotherapy, intended to kill off any malignant cells that have spread away from the primary tumor site.
10% to 20% of all cancer patients are initially misdiagnosed. Chances are good that the misdiagnosis rate for patients with Ewing’s sarcoma is far higher, since the disease is inherently difficult to identify and few specialists, let alone general practitioners, have experience interpreting the signs. An improper diagnosis can be fatal. Even when patients eventually receive the correct diagnosis, many have lost out on crucial months of treatment that could have drastically improved their prognoses and, ultimately, extended their lives even further.
But not every cancer misdiagnosis is right for a medical malpractice lawsuit. While every case is different, the majority of malpractice lawsuits rely on a theory of medical negligence, arguing that a given healthcare professional violated accepted medical standards (in diagnosis, treatment or both), which led to patient harm. Defining the appropriate standard of care in your child’s case, and then identifying how your physician violated it, will be crucial in court.