Ewing's Sarcoma

Original Editors - Lisa Miville from Bellarmine University's Pathophysiology of Complex Patient Problems project.

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Definition/Description

Ewing’s Sarcoma is a high grade malignant primary tumor that can arise in soft tissue or bone. The tumor is named after James Ewing who first discovered the small, blue round cell that was distinctly different from osteogenic sarcoma. It is often referred to as a family of tumors known as Ewing family of tumors (EFT).^[1][2][3][4] The Ewing family of tumors include Ewing’s Sarcoma, Extraosseous Ewing Sarcoma, Askin Tumor, and Primitive Neuroectodermal Tumor (source). Although any bone can be involved, the most common are the pelvis, hip, femur, tibia and fibula.^[5] Less common sites include the ribs and vertebral column.^[3] The most common sites for secondary tumors include the lungs, bone marrow and other bones; rarely does Ewing’s Sarcoma metastasize to the lymphatic system, brain or liver.

Prevelance

Ewing's Sarcoma is the second most common primary malignant bone tumor of children and the fourth most common overall. 80% of these tumors occur in people under the age of 20.  

• Youngest reported case of EFT - 5 months old 
• Oldest reported case - 77 year old female
• Rarely occurs in the African American or Asian population
• More common in males than females 1.5:1

 

Characteristics/Clinical Presentation

Pain at the affected site is usually the intial symptom. The pain may be worse during exercise or at night and can be

File:Ewing.png

Ewing's Sarcoma of left femur

accompanied by swelling or a lump, redness, and warmth (see picture below).  The pain is typically intermittent. 

 It is common to see fatigue, weight loss, decreased appetite, and/or paralysis or incontinence (if the tumor is of spinal origin).The tumor may be present for months before there are any signs or symptoms. 

An injury is often what brings attention to the tumor, because bone weakened by disease can break with little force.  Kids with EFT can go undiagnosed until an injury from a sport or rough play requires diagnostic imaging.

   

Medications

Chemotherapy medications most commonly used: vincristine (Oncovin), dactinomycin (Actinomycin D), cyclophosphamide (Cytoxan), ifosfamide (Ifex), etoposide (VePeside, VP-16), and doxorubicin (Adriamycin).

 Immuniological approaches, such as the use of cytokines (interleukins, and interferon), are still being researched.

Diagnostic Tests/Lab Tests/Lab Values

In order to properly diganose and treat Ewing's Sarcoma many images and tests need to be aquired.  The following are often used in combination for the best results: CT scan, bone scan, biopsy to see if there is bone marrow involvement, X-ray, MRI, chest scan, brain MRI or brain scan, and a blood test. It is important to scan the entire body to know whether the tumor is localized or difuse and to ensure removal of all metastatic lesions.

Ewings Sarcoma Femur seen in radiograph. Proximal portion of femur on left image

  

Etiology/Causes

It has been found that 95% of Ewing's tumors are derived from a specific genetic translocation between chromosomes 11 and 22.  The molecualr oncogenesis remains unknown.

Systemic Involvement

Ewing's Family of Tumors are highly malignant. It most commonly spreads to the lungs, but it can also metastasize to the kidney, bone marrow, heart, adrenal gland, and other soft tissues.[9] Chemotherapy and radiation, which are most commonly used to treat EFT, have many systemic side effects including hair loss, nausea, vomiting, ulcers, and low blood cell count. [9]

Medical Management (current best evidence)

Depending upon location of the tumor and metastases, doctors in many specialties help treat EFT. Medical management is considered a multidisciplinary effort which includes orthopedic surgical oncologists, pediatric or adult medical oncologists, radiation oncologists, pathologists, and radiologists. Most patients are treated at major hospital institutions or cancer centers.[3][4][10]
The first line of recommended treatment is chemotherapy, also referred to as cytostatic drug therapy, which is given through an indwelling intravenous catheter. Chemotherapy medications most commonly used are vincristine (Oncovin), dactinomycin (Actinomycin D), cyclophosphamide (Cytoxan), ifosfamide (Ifex), etoposide (VePeside, VP-16), and doxorubicin (Adriamycin).[4][7][10] Chemotherapy treatment is typically performed in cycles to let the blood cell count recover. The second line of treatment, which can be done before or during chemotherapy, is local treatment. Local treatment includes radiation and/or surgery. Surgery is used to treat the localized tumor when the tumor is easily assessable. When the localized tumor is not assessable as in the pelvis or spine, surgery is not an option and radiation is used to treat the localized tumor. A detrimental side effect that can result from radiation is structural deformities in children. [17] Surgery can also be performed to rebuild a body part or limb. As the child grows, reconstruction therapy will be necessary to lengthen the bone.[3][4][10]
Follow up intervals of 2-4 months for the first 3 years after completion of therapy are recommended for high-grade tumors such as EFT. Follow up every 6 months for year 4 and 5 and annually after that.[3] Due to recent availability of multi-agent cytostatic approaches and local therapy, the 5 year survival rate has increased from 10% to 70%.[4][6]
Immunological approaches, such as the use of cytokines (interleukins, and interferon), are still being researched. [15]

Physical Therapy Management (current best evidence)

Physical therapy (PT) can be beneficial for those diagnosed with EFT for many reasons and at different stages during the management process. The most common limitations for patients undergoing chemotherapy include fatigue, paralysis, or weakness, cognition, and weight loss/ gain [5].
Pre-operative PT is beneficial when feasible. Plan of care should include strengthening of affected limb and aerobic conditioning (precaution: avoid weight bearing on extremity or placing weight distal to extremity in which tumor is located).[12]
Post-operative PT is essential but caution must be taken because of the impaired healing process due to chemotherapy. Precautions include: stretching the skin in the area of incision, weight bearing status and lab values (especially platelet count). Some general guidelines include: aerobic conditioning, strengthening, continuous passive range of motion, and aquatic therapy.[12]
If amputation is done, it may take the child several months to learn to use a prosthetic leg or arm. A physical therapist will be able to assist in fitting and donning the prosthesis, teaching the child how to use it, and how to use necessary assistive devices. Children may also have a tissue graft, which the child needs to start moving almost immediately. Physical therapy and rehabilitation is typically recommended for six to twelve weeks post operation.[10]

Differential Diagnosis

Osteomyelitis and Ewing's Sarcoma may both present with swelling, redness, low grade fever, and flu-like symptoms. The patient will typically experience intermittent pain with Ewing's Sarcoma versus the constant pain seen with osteomyelitis. The patient may also experience extreme fatigue, weight loss, and loss of appetite with a tumor. Antibiotics will not affect Ewing's Sarcoma, but should help with osteomyelitis.[5] X-rays and Lab values are utilized to distinguish Ewing’s Sarcoma and Osteomyelitis. Ewing’s Sarcoma’s symptoms are very general and a differential diagnosis of this condition include tendonitis, Osgood Schlatter disease, trauma, and slipped epiphysis of a long bone. [13]

Review an excellent Presentation on Ewings Sarcoma by Jen Son and Gilian Lieberman MD

      

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Case Reports/ Case Studies

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References

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