Ewing's Sarcoma
Original Editors - Lisa Miville from Bellarmine University's Pathophysiology of Complex Patient Problems project.
Lead Editors - Your name will be added here if you are a lead editor on this page. Read more.
Definition/Description[edit | edit source]
Ewing’s Sarcoma is a high grade malignant primary tumor that can arise in soft tissue or bone. It is often referred to as a family of tumors known as Ewing family of tumors (EFT).[1][2][3][4] The tumor is named after James Ewing who first discovered the small, blue round cell that was distinct from osteogenic sarcoma.[4] Although any bone can be invovled, the most common are the pelvis, femur, tibia, ulna and metatarsals.[5] Close behind is the ribs and vertebral column.[3] EFT is most common in children between the ages of 5 and 16, and 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] Prognosis is dependent on metastisis, worsening with widespread involvement. 26% of patients with EFT have metatstic disease: 10% to the lungs, 10% to bone/bone marrow, and 6% combination.[3]
Prevelance[edit | edit source]
Ewing's Sarcoma is the second most common primary malignant bone tumor of children and the fourth more common overall. 80% of these tumors occur in people under the age of 20. They have been reported in children as young as 5 months and the oldest person was 77. They rarely occur in the African American or Asian population. EFT is more common in males than females 1.5:1.[7][2][3][4][6][8]
Characteristics/Clinical Presentation[edit | edit source]
Pain at the affected site is usually the intial symptom.[4] The pain may be worse during exercise or at night and can be accompanied by swelling, redness, and warmth (see picture below).[9] The pain is typically intermittent.[5] It can also be common to see fatigue, weight loss, decreased appetite, and/or paralysis or incontinence (if the tumor is of spinal origin).[10]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.[11]
Associated Co-morbidities[edit | edit source]
Pathological Fractures secondary to osteomalacia.
Medications[edit | edit source]
Chemotherapy medications most commonly used: vincristine (Oncovin), dactinomycin (Actinomycin D), cyclophosphamide (Cytoxan), ifosfamide (Ifex), etoposide (VePeside, VP-16), and doxorubicin (Adriamycin).[4][8][11] Immuniological approaches such as the use of cytokines (interleukins, and interferon) is still being researched.[4]
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
Chemotherapy, radiation, surgical resection, limb salvage, amputation.[7][2]
Etiology/Causes[edit | edit source]
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.[7][4][11]
Systemic Involvement[edit | edit source]
EFT is a highly malignant tumor. It most commonly spreads to the lungs, but it can also metastisize to the kidney, bone marrow, heart, adrenal gland, and other soft tissues.[10] 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.
Medical Management (current best evidence)[edit | edit source]
Depending upon location of tumor and metatstasis, doctors in many specialties help treat EFT. These include 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][11]
The first line of treatment is chemotherapy, also referred to as cytostatic drug therapy, which is given through an indwelling intravenous catheter. It is done in cycles to let the blood cell count recover inbetween. The second line of treatment, which can be done before or during chemotherapy, is local treatment. Local treatment includes radiation and/or surgery.Radiation can be used instead of surgery if it is too risky or complicated. It decreases the chance of return of the primary tumor. Over serveral weeks, daily treatments of radiation are given.Surgery can be done to remove the primary tumor or metastatic lesions. It can also be done to rebuild a body part or limb. As the child grows, reconstruction therapy will be necessary to lengthen the bone.[3][4][11]
Follow up intervals of 2-4 months for the first 3 years after completeion 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]
Physical Therapy Management (current best evidence)[edit | edit source]
Physical therapy (PT) can be beneficial for victim's of EFT for many reasons and at different stages during the management process.
Pre-operative PT is beneficial when feasible.It 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 opereative 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, lab values (especially platelet count). Soem general guidlines include: aerobic conditioning, strengthening, continuous passive range of motion, and aquatic therapy.[12]
lIf 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 the prosthesis, teaching the child how th 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.[11]
Alternative/Holistic Management (current best evidence)[edit | edit source]
Although there plenty of resources describing the use of diet and herbal remedies for curing cancer, there is not sufficient evidence or reasearch to support these treatments.
Differential Diagnosis[edit | edit source]
Osteomyelitis - swelling, redness, low grade fever and flu-like symptoms may be present in both conditions.
Case Reports/ Case Studies[edit | edit source]
add links to case studies here (case studies should be added on new pages using the case study template)
Resources
[edit | edit source]
Recent Related Research (from Pubmed)[edit | edit source]
see tutorial on Adding PubMed Feed
Failed to load RSS feed from http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1dI9jD-j4R7UTpQM4CIWX4TqTsuoDek2xQeiSGlysd_19VhQDN|charset=UTF-8|short|max=10: Error parsing XML for RSS
References[edit | edit source]
see adding references tutorial.
- ↑ Pathology Book
- ↑ 2.0 2.1 2.2 Medline Plus. Ewing’s Sarcoma. http://www.nlm.nih.gov/medlineplus/ency/article/001302.htm (accessed Feb 2011).
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Grimer, Robert, Athanasou, Nick, Gerrand, Craig, Judson, Ian, Lewis, Ian, Morland, Bruce, Peake, David, Seddon, Beatrice, Whelan, Jereamy. UK Guidelines for the Management of Bone Sarcomas. Sarcoma 2010; 1-14. http://www.hindawi.com/journals/srcm/2010/317462/ (accessed 28 Feb 2011).
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 4.9 Paulussen, Michael. Frohlich, Brigit, Jurgens, Herbert. Ewing Tumour: Incidence, Prognosis, and Treatment Options. Pediatric Drugs 2001; 3(12); 899-913. http://www.ncbi.nlm.nih.gov/pubmed/11772151 (accessed 28 Feb 2011).
- ↑ 5.0 5.1 Elaine Lonneman’s Powerpoint, Oncology. Pathophysiology of Complex Patient Problems. Bellarmine University 2011.
- ↑ 6.0 6.1 Goodman C, Snyder T. Differential Diagnosis for Physical Therapists: Screening for Referral. St. Louis, MO: Saunders Elsevier: 2007
- ↑ 7.0 7.1 7.2 Cite error: Invalid
<ref>tag; no text was provided for refs namednull - ↑ 8.0 8.1 Karosas, Ann O. Ewing’s sarcoma. Am J Health-Syst Pharm 2010; 67; 1599-1605. http://www.ajhp.org/content/67/19/1599.full.pdf+html (accessed 28 Feb 2011).
- ↑ Kids Health from Nemours. Ewing Sarcoma. http://kidshealth.org/parent/medical/cancer/ewings.html (accessed 28 Feb 2011).
- ↑ 10.0 10.1 Children’s Hospital Boston. Ewing’s Sarcoma. http://www.childrenshospital.org/az/Site852/mainpageS852P0.html (accessed March 2011)
- ↑ 11.0 11.1 11.2 11.3 11.4 11.5 American Academy of Orthopaedic Surgeons. Ewing’s Sarcoma. http://orthoinfor.aaos.org/topic.cfm?topic=a00082 (accessed 28 Feb 2011).
- ↑ 12.0 12.1 Tiley, Karen L. Multidisciplinary Rounds: Limb Salvage Surgery and Bone Marrow Transplant in an Adolescent with Recurrent Ewing’s Sarcoma. Cancer Practice 1995; 3(2); 72-75.
