Ewing's Sarcoma Radiologic Findings by pengxiuhui

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									Ewing’s Sarcoma

 Paul Naumann MS4
History of Ewing’s Sarcoma

• James Ewing originally described it in
  1921.
• Ewing first described the tumour as an
  "endothelioma of bone" believing that it
  arose from the blood vessels of bone
  tissue.
History of Ewing’s Sarcoma

• He later recognised that the histopathologic
    features were more complex and went on to
    describe it as a "endothelial myeloma"
•   He described the histopathology as "broad
    sheets of small polyhedral cells with pale
    cytoplasm, small hyperchromatic nuclei, well-
    defined cell borders and complete absence of
    inter-cellular material"
 Epidemiology/Pathology
 • Ewing's sarcoma is an unusual disease
      comprising about 4-6% of all primary bone
      tumors.
 •    Although it has been reported as occurring at all
      ages, by far the majority develops within the
      first two decades of life. In the second decade, it
      is the most common primary neoplasm of bone
      after osteosarcoma.
 •    The usual site of occurrence is in the diaphysis
      of long bones, less often in ribs, pelvis and
      vertebrae [1]
1. Hardy P, Gibbs AR. Ewing's sarcoma of mandible: British Journal of Oral and Maxillofacial Surgery 1984; 22: 287-
94.
 Epidemiology/Pathology
 • Involvement of the head and neck in Ewing's
      sarcoma is very unusual, accounting for
      approximately 1% to 4% of cases [2].
 •     Males are affected somewhat more commonly
      than females; the ratio is 3:2.
 •    Unlike osteosarcoma, Ewing's sarcoma has a
      predilection for whites; blacks and Asians are
      uncommonly affected [3]

1. Fonseca AS, Mezzalira R, Crespo AN, Bortoleto AE Jr, Paschoal Jr. Ewing's sarcoma of the head & neck: Sao Paula
Med J 2000 Nov; 118(6): 198-200.
2. Fielding AF, Lindemeyer R, Wood-Hartman MJ. Ewing's sarcoma of the mandible: combined approach to
treatment: J Clin Pediatr Dent 2002 summer; 26(4): 409-12.
What are the symptoms of
Ewing's sarcoma ?

• Symptoms of Ewing's sarcoma vary from person to
    person and depending on the location and size of the
    cancer.
•   The most common symptoms are pain and swelling or
    tenderness in the affected area.
•   Pain may become very intense when the tumour is
    located near important nerves, like in the sacrum, pelvis
    or spine.
•   Swelling is often seen, especially when the log bones of
    the arms or legs are affected.
Ewing Sarcoma Family of Tumors
(ESFTs)
• Includes:
  – Ewing tumor of bone.
  – Extra osseous Ewing.
  – PNET
Diagnosing of ESRT

• Expression of CD99 surface protein.
• More than 90% of ESFTs have an 11,22
  translocation.
• PNETs are distinguished from other types
  of Ewing sarcoma by evidence of neural
  differentiation.
 National Cancer Institute. Ewing’s family of
 tumors (PDQ): treatment. Available at
 www.cancer.gov. Accessed November 19,
 2002.
Primary tumor sites in a series of
900 people with Ewing’s
Staging of Ewing’s Sarcoma
• At present, there is no formal staging system for
    Ewing's sarcoma/primitive neuroepithelial tumor.
•   Patients are grouped depending on whether
    cancer is found in only one part of the body
    (localized disease) or whether cancer has spread
    from one part of the body to another (metastatic
    disease).
Staging of Ewing’s Sarcoma
• Localized
  The cancer cells have not been shown to have spread beyond the
  bone in which the cancer began or are found only in the bone and
  nearby tissues.
  Metastatic
  The cancer cells have spread from the bone in which the cancer
  began to other parts of the body. The cancer most often spreads to
  the lung, other bones, and bone marrow (the spongy tissue inside
  of the large bones of your body that makes red blood cells). Spread
  of cancer to the lymph nodes (small bean-shaped structures found
  throughout your body which produce and store infection-fighting
  cells) or the central nervous system (brain and spinal cord) is less
  common.
  Recurrent
  Recurrent disease means that the cancer has come back (recurred)
  after it has been treated. It may come back in the tissues where it
  first started or it may come back in another part of the body.
Treatment of Ewing’s Sarcoma
• Localized Ewing's sarcoma/primitive neuroepithelial tumor
  Treatment for localized Ewing's sarcoma/primitive neuroepithelial tumor
  depends on where the cancer is found in the body.
  If the cancer is in the bone below the elbow or knee or in the jaw, skull,
  face, shoulder blade, collar bone, or segments of the spinal column,
  treatment may be one of the following:
  1. Combination chemotherapy
  2. Surgery and combination chemotherapy.
  3. Radiation therapy and combination chemotherapy.
  4. A clinical trial of chemotherapy and new ways of giving radiation therapy.
  5. A clinical trial of chemotherapy followed by surgery, with or without
  radiation therapy.
  6. Clinical trials are also evaluating new doses and combinations of
  chemotherapy.
Treatment cont….
• Metastatic Ewing's sarcoma/primitive neuroepithelial tumor
  Treatment may be one of the following:
  1. Combination chemotherapy.
  2. Radiation therapy plus combination chemotherapy.
  3. Combination chemotherapy plus surgery to remove cancer that
  has spread to the lungs.
  4. Clinical trials are evaluating new doses and combinations of
  chemotherapy with or without radiation treatment.
Case

• 25 yo female.
• Biopsy proven Ewing sarcoma of the right
  5th metatarsal.
• Received 6 rounds of chemo prior to
  amputation as part of Children’s Oncology
  Group study protocol.
Findings:
1. An expansile lytic lesion of
distal the 5th metatarsal.
2. Cortical thinning.
Findings:
1. Cortical thinning.
2. Soft tissue inflammation vs.
   tumor invasion.
Findings:
1. Marrow invasion, signal similar
to muscle tissue.
Linear signal representing
extra cortical
involvement.
Post amputation radiographs.
Thanks

								
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