Ewing's Sarcoma

Original Editors - Lisa Miville <a href="Pathophysiology of Complex Patient Problems">from Bellarmine University's Pathophysiology of Complex Patient Problems project.</a>

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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).^{Goodman, Boissonnault, Fuller. Pathology: Implications for the Physical Therapist. Pennsylvania: Saunders, 2003.Medline Plus. Ewing’s Sarcoma. http://www.nlm.nih.gov/medlineplus/ency/article/001302.htm (accessed Feb 2011).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).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).} The Ewing family of tumors include Ewing’s Sarcoma, Extraosseous Ewing Sarcoma, Askin Tumor, and Primitive Neuroectodermal Tumor. Although any bone can be involved, the most common are the pelvis, hip, femur, tibia and fibula.^{Elaine Lonnemann’s Powerpoint, Oncology. Pathophysiology of Complex Patient Problems. Bellarmine University 2011.} Less common sites include the ribs and vertebral column. 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.Goodman C, Snyder T. Differential Diagnosis for Physical Therapists: Screening for Referral. St. Louis, MO: Saunders Elsevier: 2007

Prevelance

Ewing’s Sarcoma primarily affects Caucasians, whereas Asian and African populations are considerably less affected . Rates of Ewing’s Sarcoma are found to be higher in North America as compared to Europe or Asia. The rate of prevalence between males and females is not significantly different. Ewing’s Sarcoma is not currently related to any gene mutations or hereditary cancer syndromes. However, relationships between race and inherited familial risk suggests a possible association. 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. EFT is most common in children between the ages of 5 and 16. Factors include a tall stature or experiencing a premature growth spurt from puberty. In adults risk factors such as infection, radiation, and occupation increase the risk of development of Ewing’s Sarcoma.Burningham Z, Hashibe M, Spector L, Schiffman JD. The Epidemiology of Sarcoma. Clinical Sarcoma Research. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3564705/. Published 2012.

• Youngest reported case of EFT - 5 months old 
• Oldest reported case - 77 year old female
  
• More common in males than females 1.5:1

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). 

Incidence

The incidence of Ewing’s Sarcoma has relatively been unchanged for the past 30 years, and occurs 1 per 1 million people in the United States. Currently in the United States, the incidence rate is currently nine fold greater in Caucasian males and females than in African Americans, and intermediate recurrence in Asian Americans. 16

Prognosis

Ewing’s sarcoma is typically cured 70% of the time in the early stages of metastasis. Teenagers in the range of 15-19 have a lower survival rate of 56%. 17 After the disease has spread, children have a survival rate of less than 30%. 17

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 molecular oncogenesis remains unknown.

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<img src="/images/c/c4/Ewing.png" _fck_mw_filename="Ewing.png" _fck_mw_type="frame" alt="Ewing's Sarcoma of left femur" class="fck_mw_frame fck_mw_right" /> accompanied by swelling or a lump, redness, and warmth (see picture below).Kids Health from Nemours. Ewing Sarcoma. http://kidshealth.org/parent/medical/cancer/ewings.html (accessed 28 Feb 2011).  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).Children’s Hospital Boston. Ewing’s Sarcoma. http://www.childrenshospital.org/az/Site852/mainpageS852P0.html (accessed March 2011)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.American Academy of Orthopaedic Surgeons. Ewing’s Sarcoma. http://orthoinfor.aaos.org/topic.cfm?topic=a00082 (accessed 28 Feb 2011).

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

http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1VsHRGSo3HX0CgCh0yQTDe7FwEqMdcz0ZoLhJtVqvvv0fjjM7M%7Ccharset=UTF-8%7Cshort%7Cmax=10

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]

Diagnostic Tests/Lab Tests/Lab Values

When diagnosing Ewing’s Sarcoma the first test most commonly used is X-Ray over the painful area. Other tests that may be performed to rule in Ewing’s Sarcoma and determine staging are: bone scans, CT scans, MRI, blood tests (elevated lactate dehydrogenase and red blood cell sedimentation rate) and biopsy. These tests in combination are important to find the location of the tumor and to determine if the tumor is localized or has diffused to other areas of the body to help guide treatment. Biopsy is considered the gold standard test for diagnosis of Ewing’s Sarcoma.[1][11][3]

 <img src="/images/d/df/Xrayewings.jpg" _fck_mw_filename="Xrayewings.jpg" alt="" />

<img src="/images/thumb/8/84/EwingsSarcomaBU.jpg/400px-EwingsSarcomaBU.jpg" _fck_mw_filename="EwingsSarcomaBU.jpg" _fck_mw_location="center" _fck_mw_width="400" _fck_mw_type="border" alt="Ewings Sarcoma Femur seen in radiograph. Proximal portion of femur on left image" class="fck_mw_border fck_mw_center" />  

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]

                                    

Ewing’s Sarcoma Case Study

Keywords

Fatigue, treatment, fever, bone pain, cancer, therapy, symptoms

Authors

Jack Tencza and Joseph Dorrell

Abstract

Ewing’s Sarcoma is the 2nd most commonly diagnosed form of primary bone cancer in children and young adults. In this example, a case study of an 18 year old Caucasian female gymnast is examined to help health care professionals identify a possible clinical case of Ewing’s Sarcoma.

Introduction

Ewing’s Sarcoma family of tumors are a group of small round cell tumors that include Ewing’s Sarcoma, Extraosseous Ewing Sarcoma, Askin Tumor, and Primitive Neuroectodermal Tumor. This cancer primarily affects children and adolescents, and most often affects soft tissue and bone. The most common sites for Ewing’s Sarcoma is the pelvis, hip, femur, tibia and fibula.

Case Presentation

Subjective :

Patient History: Pt. is an 18 year old Caucasian female who reports with a recent history of right hip pain of 5/10 during the day and 7/10 at night. She reports limping from a cartwheel at gymnastics 2 months ago. She reports that she has had night sweats with intermittent fever and general fatigue during the day. Pt. reports that she has had a recent physical examination 5 months ago with her GP who found that she was underweight. GP states this could be possibly due to female athlete triad. She notes that she has been more attentive with her diet and exercise since then. Pt. states her goal is to return to gymnastics pain free and as soon as possible.

Medical History: Unremarkable

Objective : Physical Examination Tests and Measures

Observation/ Palpation: Pt. has pinpoint tenderness around R ASIS, and notes pain with R weight shifting

Lumbar ROM
Lumbar Flexion 65
Lumbar Extension 35
Lumbar Sidebend 20
Lumbar Rotation 30

Hip ROM Right Left
Hip Flexion 85 130
Hip Extension 15 30
Hip IR 20 40
Hip ER 25 40
Hip ABD 25 45

MMT
L Hip 5/5 in all planes
R Hip 4/5 in all planes (pain)
UE ROM: WNL
UE MMT: WNL

Neurovascular: decreased sensation along R lateral thigh

Special Tests:
FABER - Negative
Scour Test - Positive
Anterior Labral Tear - Negative
Impingement Test - Positive

Clinical Impression

Examination findings show decreased ROM and weakness of the R hip with palpable pinpoint pain, tenderness, and warmth. No other musculoskeletal abnormalities found to be present. Working diagnosis of female athlete triad and hip impingement. Targeted interventions include strengthening the R hip musculature, improving R hip ROM in all planes, manual therapy to decrease pain and improve function, and modalities for pain relief.

Intervention

Hip AROM/ PROM exercises
Standing hip flexion, extension, abduction, IR and ER resistance exercises
Wall slides & standing squats
Hip long axis traction

Outcomes

Over a period of 5 visits pt’s s/s did not improve. Pain intensified to a 7/10 during the day and an 8/10 at night. PT then determined that pt needed to be referred out for imaging of the hip. Pt went to MD and received X-Ray which indicated a possible tumor of the pelvis. Blood tests indicated elevated lactate dehydrogenase and red blood cell sedimentation rate. MD referred to Oncologist for biopsy which confirmed a Ewing’s Sarcoma of the pelvis. Pt received chemotherapy and radiation.

Discussion

Ewing’s Sarcoma is a malignant bone tumor which in this case affects a female, which is not as common as a male. In this case, the pt. demonstrated constitutional signs and symptoms including night sweats, weakness, fatigue, intermittent fever and increased pain at night which could indicate a systemic problem. Physical therapists should be aware that Ewing’s Sarcoma can mimic musculoskeletal signs and symptoms which can make it difficult to diagnosis. It is imperative to monitor the patient and refer out to patient’s MD when appropriate to prevent further metastasis of the tumor. The pelvic and hip region is the most common area affected by Ewing’s Sarcoma and can be difficult to observe and palpate due to its location. In this case the pt did not improve upon multiple visits which would indicate it is not a musculoskeletal pathology and the pt. was referred to her primary care provider for further testing.

Related Pages

Halwai MA, Mir BA, Wani MM, Bashir A, Hussain A. Ewing's sarcoma of the ilium mimicking inflammatory arthritis of the hip: a case report. Cases Journal. 2009;2(1):6487. doi:10.4076/1757-1626-2-6487. http://casesjournal.biomedcentral.com/articles/10.4076/1757-1626-2-6487

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References

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