Ewing’s sarcoma is an aggressive type of bone and soft tissue cancer that primarily affects children and teenagers. The prognosis is good if the cancer is caught in the early stages before it has spread. Fortunately, it is a rare cancer, occurring in approximately one out of every million people in the United States. However, 10 cases per million people include adolescents between the ages of 10 and 19, resulting in 200 cases diagnosed annually. Here is an overview of the signs and symptoms of Ewing’s Sarcoma.
There are two main types of cancer, carcinoma and sarcoma. The former is the most common type of cancer. Carcinomas develop in the epithelial cells, which surround internal organs and line the surfaces of the body, including the skin, urinary tract, and blood vessels.
These cells provide a protective barrier against outside forces, such as bacteria and viruses. Epithelial cells are shaped like columns when viewed under a microscope. Carcinomas typically affect the mucous membranes and skin. Basal cell, squamous cell, transitional cell carcinomas and adenocarcinoma are the most common types of epithelial cancers.
Sarcomas develop in the mesenchymal cells in the bones and soft tissues, including the tendons, muscles, and blood vessels. Mesenchymal cells are multipotent stromal cells that have the ability to differentiate into other types of cells. These cells include osteoblasts in the bone, myocytes in the muscles, chondrocytes in cartilage, and adipocytes in fat cells. Stromal cells are connective tissues cells within the organs, including the lymph nodes, prostate, ovary, and uterus.
Sarcoma tumors develop in the bone, cartilage, joints, tendons, ligaments, muscles, fat, nerves, and blood vessels. There are over 75 types of sarcomas, with most occurring in the legs, arms, and abdomen. 87% of Ewing’s sarcoma occurs in the bones. Approximately 90% of all types of cancer are carcinoma, with sarcoma being much more rare.
Other types of sarcomas include undifferentiated pleomorphic sarcoma, which develops simultaneously in the bones and soft tissue, leiomyosarcoma, cancer of the smooth muscles cells in the GI tract, uterus, and blood vessels, lipsosarcoma that develops in fat cells, and osteosarcoma, cancer of the bone cells. Peripheral primitive neuroectodermal tumor (pPNET) is a type of tumor that develops in the nerves in various parts of the body.
How Tumors Develop
The human body is made up of trillions of cells. These cells are constantly growing, dividing, and replacing old, damaged cells. Cancer is the result of this process no longer functioning as it should. When old cells are no longer replaced by new cells, they can divide uncontrollably to form tumors.
Tumors are abnormal growths of tissue, or malignant masses, caused by the proliferation, division, and spread of cancer cells. Tumors can be deadly because as they increase in size, malignant cells can break off from the tumor and travel to other locations in the body via the lymph system or blood stream. New tumors can then form in new locations, which may be far removed from where the original tumor developed.
Cancer cells can negatively affect normal cells, blood vessels, and molecules that surround a tumor, enabling its growth by creating new blood vessels. This process is called angiogenesis. New blood vessels feed the existing tumor by supplying it with nutrients and oxygen, allowing it to grow and thrive. Waste is also removed by these new blood vessels.
Metastatic cancer is cancer that has spread from its original location. Advanced cancers are referred to by the same name as the original cancer. For instance, breast cancer that has spread to the bones or lungs is not called bone or lung cancer, but rather metastatic breast cancer. This is because the malignant cells look the same as the original cancer cells.
Ewing’s Sarcoma Diagnosis
Physical exams are used to diagnose Ewing’s sarcoma, along with various other diagnostic testing methods, such as biopsies, bone marrow aspirations, bone scans, X-rays, CT and PET scans, MRIs and blood tests. What testing methods are used depends on several factors, including the type of cancer that is suspected, the location and size of the tumor, if metastases is present, the age of the patient and the symptoms they’re experiencing, along with the general state of their health and the results of previous testing.
A biopsy is a definitive way to determine if cancer is present in the suspected area. Small tissue samples are taken from the area of growth and sent to a lab for evaluation. Children are typically put under anesthesia for the procedure. If for some reason a biopsy is not able to be taken, other testing methods are used. Blood tests, notably CBC or complete blood counts, analyze red and white blood cells and platelets. Cell abnormalities are red flags for tumor growth and metastases.
Bone marrow aspiration, combined with biopsy, removes cells from the bone marrow to check for cancer. The procedure is performed under a local, quick-acting anesthetic that is injected into the skin to reduce pain, with the option to use general anesthesia instead. The liquid portion of the bone marrow is removed during an aspiration, with a biopsy immediately following. A biopsy is performed, in case the bone marrow that was extracted during the aspiration, did not contain enough malignant cells to make a diagnosis.
Bone Scans and Imaging Tests
Imaging tests, CT scans, PET scans, and MRIs, take images of internal organs to look for tumors or other abnormalities, along with their size. Specifically for Ewing’s Sarcoma, CT scans of the chest are used to see if there is metastases in the lungs. X-rays use a small amount of radiation to take pictures of tissues and organs to detect bone tumors.
Bone scans are nuclear imaging tests used to diagnose bone tumors and metastatic bone cancer. Small amounts of a radioactive tracer is injected into a vein. Areas of repair in the body take up the most amount of tracer. A radiologist then evaluates the scan for abnormalities in bone metabolism. Areas of bone that contain a tumor appear dark, and are referred to as “hot spots,” whereas healthy bone is gray.
Causes and Treatment
Ewing’s sarcoma is not an inherited cancer, how it develops is unknown. The disease, does however, correlate to changes seen in certain non-inherited genes that can occur during a person’s lifespan. Two chromosomes, specifically chromosomes 11 and 12, have the ability to exchange genetic material. This exchange can stimulate cell overgrowth, potentially leading to Ewing’s sarcoma. It’s also surmised that Ewing’s sarcoma may be a secondary cancer in people who have been treated with radiation for a previous cancer.
How bone cancer is treated depends on the location and size of the tumor and whether it has spread. While there is no standardized treatment for Ewing’s sarcoma, conventional options include surgery, chemotherapy, radiation, stem cell transplants, and targeted proton therapy. Some patients choose to incorporate alternative cancer therapies into their treatment protocols. Immunotherapy, stem cell transplants, and monoclonal antibody therapy are used for metastasized bone cancer.
Signs and Symptoms Of Ewing’s Sarcoma
In 1920, Dr. James Ewing, an American pathologist, discovered a type of malignant bone tumor that develops in soft tissue and bone, notably, the ribs, hip bones, shoulder blades, and long bones, including the femur, tibia, and humerus in the upper arm. It’s common for sarcomas to initially develop in the pelvis or thighbone. Sarcomas can metastasize to other locations in the body, namely the lungs, adrenal glands, liver, bone marrow, kidneys, heart and other soft tissues.
Although, Ewing’s sarcoma can occur at any age, it commonly develops during puberty. This is most likely due to rapid bone growth during this time. More males are affected by the disease than females. Interestingly, this type of cancer rarely occurs in those of African or Chinese descent. Caucasians are nine times more likely to be diagnosed with Ewing’s sarcoma.
Pain and swelling in the area of tumor growth are the most common symptoms of bone cancer. The area may be warm to the touch and develop a lump on the surface. Other symptoms include:
- chronic low-grade fever
- loss of appetite
- pain that worsens with exercise
- unintentional weight loss
- unexplained fatigue
- feeling unwell
- breaking a bone for no apparent reason
Sarcomas that develop in the pelvis, chest, arms or legs, may have corresponding symptoms related to that area. For example, a person may have difficulty breathing if the tumor is in the chest area. Treatment is less successful for cancer that has spread, with five-year survival rates hovering around 70%.
Like any type of cancer, Ewing’s sarcoma can be extremely frightening, and particularly so because it occurs in young people. It is the second most common type of childhood cancer, and is responsible for one percent of all childhood cancers. It is rare in adults.
Ewing’s sarcoma is found in the long bones of the body, but can develop in any bone, along with the soft tissue and muscle surrounding the tumor. If you or someone you love has any of the symptoms above, please schedule an appointment with your doctor so the cancer can be detected and treated in the early stages for the most favorable prognosis.
Do you know someone who has Ewing’s Sarcoma? Let me know in the comments:)
(1) WebMD: What Is Ewing’s Sarcoma?
(2) healthline: What Is Ewing’s Sarcoma?
(3) National Cancer Institute: What Is Cancer?
(4) Mayo Clinic: Bone scan
(5) Cancer.Net:wing Sarcoma – Childhood and Adolescence: Diagnosis
(6) Memorial Sloan Kettering: Ewing Sarcoma Diagnosis
(7) John Hopkins Medicine: Ewing Sarcoma in Adults
(8) NCBI: Promiscuous Partnerships in Ewing’s Sarcoma
(9) NCBI: Current Therapeutic Approaches in Metastatic and Recurrent Ewing Sarcoma
(10) UCSF Benioff Children’s Hospital: Ewing’s Sarcoma Diagnosis
Disclaimer: This article is strictly for informational purposes only and is not intended to be medical advice.