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Ewing's sarcoma
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Ewing's sarcoma

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  • 1. Ewings Tumor
  • 2. History: 1928 James Ewing described a rare lesion Diaphysis of long bones Childhood Febrile attacks,anorexia, weight loss & anaemia Rapidly involving other parts of skeleton Radiosensitive Histologically –endothelial elements in marrow
  • 3.  Willis – All these tumors were not Ewing’s but Neuroblastomas Reticulum cell sarcomas Metastatic tumors
  • 4. Ewing’s Sarcoma Epidemiology: 4th. m.c. primary malignancy of bone 2nd. m.c. in age < 30 yrs. Incidence < 1/ million / year 9% of primary malignancies of bonein the Mayo Clinic series.
  • 5.  Age – Wide age range from infants to elderly m.c. 5 – 15 yrs. Male slightly more than females. Very rare in Africans
  • 6. Location: M.c. in metaphysis of long bones ( often extending intodiaphysis), Flat bones of shoulder and pelvis. Rarely in spine or small hand & footbones. Long bones Tibia > fibula > humerus > femur.
  • 7.  Spread – Whole of the skeleton. Regional lymph nodes.
  • 8. Clinical features: Pain – Universal complaint Insidious in onset, long standing, intermittentattacks, responding to analgesics. Often H/O preceding trauma. Slow growing tumor in relation to the shaft of long bones.
  • 9.  Delay in diagnosis Av. 34 weeks Patient delay – 15 weeks Physician delay 19 weeks – Importance of Xrays & rpt. Xrays to compare. During attacks of pain tumor size may enlarge visibly.
  • 10. Other clinical features: Fever, erythema & swelling ~ Osteomyelitis Skin – not involved unless surgical exploration done. Pathological # seldom. Later stage – multiple deposits in skull, ribs, sternum,pelvis & other long bones – cachexia & sec. Anaemia.
  • 11.  Vertebral involvement – severe root pain or paralysis Death – Metastatic involvement of lungs.
  • 12. Radiological appearance: X-rays: Classically Destructive lesions, diaphysislong bones, onion peelappearance. Reality Metaphysis long bones frequently extendinginto diaphysis.
  • 13.  Diffuse rarefaction towards center of shaft extendingto considerable area peripherally. Flat bones: Non-specific destructive lesions & largeadjacent soft tissue mass.
  • 14.  Early stage: Condensation Later Reactive irritation – Onion skin layers Last stage Gross tumor formation, destruction ofbone, pathological #s.
  • 15.
  • 16.
  • 17.  MRI: Taken of entire bone involved Extent of diseased bone Soft tissue involvement
  • 18. Other Investigations: Blood Ix: WBC Leukocytosis, ESR CRP LDH ~ suggestive of infective cause. FNAC – resembles Pus.
  • 19.  Base line X ray & CT chest – m.c. site of metastasis Bone Scan Bone marrow aspiration – routine R/O diffuse systemic disease USG abdomen liver & spleen.
  • 20. Pathology: Begins in - Marrow (Mid shaft) Gross – Color - Greyish-white Areas of necrosis & hemorrhage with cyst formation. Lamellae destruction.
  • 21.  Medulla Haversian canals Surface. Sub periosteal compensatory layers of new bone aredeposited only to be destroyed Onion Skinappearance.
  • 22. Histology: Microscopic features – nonspecific. Intensely cellular Cells – Usually one type Blue, Small, round & polyhedral Arrangement – solidcords or sheets.
  • 23.  Intercellular substance – minimal Necrosis Remaining cells arrange around the central bloodvessel – perithelioma. Nuclei – prominent Mitosis - frequent
  • 24.  Rossette arrangement with central fibril(Neuroblastoma) Pseudorosette (without fibril) more common. Despite bone destruction Giant cells/ osteoclasts are not found Nor new bone Except sub periostealdeposits.
  • 25.  Vessels & lymphatics Tumor emboli tumorspread.
  • 26. Cytogenetics &Immunohistochemistry: To differentiate from other small blue cell tumors. m.c. translocations in >90% cases t(11;22)(q24;q12)diagnostic of Ewing’s. Other diagnostic translocations include t(21;22)(q22;q12) & t(7;22)(p22;p12)
  • 27. IHC Staining for MIC2 gene products – Specific PAS + usually (high intracellular glycogen). Reticulin – ( c.f. lymphomas)
  • 28.  Histological D/D Lymphomas – PAS -, Reticulin +, Embryonal rhabdomyosarcoma – desmin , myoglobin,muscle specific actins + Hemangeopericytomas – Factor VIII + Small cell metastatic carcinomas & Melanomas –Cytokeratin +
  • 29. Other D/D: Chronic Osteomyelitis Metastatic Neuroblastoma Histiocytosis Rarely Osteolytic Osteosarcoma
  • 30. Treatment: Adjunct or Neoadjunct Chemotherapy or both – fordistant metastasis. Local Treatment: (Controversial) Highly radiosensitive Wide resection Decreases local recurrence to <10% Increases overall survival
  • 31.  Large, central, unresectable – Radiation Small, more accessible lesions – Surgery Choice is individual based. Repeat Staging studies after neoadjunct chemotherapy. Repeat X rays - ossification Repeat MRI - soft tissue mass.
  • 32.  At this stage – If Lesions can be resected with wide margins & anacceptable functional deficit – SURGERY. If not – RADIATION. Radiation: Marginal Resection or Contaminated wide resection.
  • 33.  Treatment Plan: After long D/W patients & parents Include expected function after amputation, limbsalvage & radiation Inherent short & long term risks with each option. Disease relapse – and its prognosis.
  • 34. Survival rates : Long term survival rates reported from most studies –60-70% Before the use of chemotherapy – 10 %
  • 35. Prognostic factors: Location & Size of primary lesion. Older age of presentation & male gender. Distant metastasis – worst prognostic factor with 20%long term survival even with aggressive therapy andsurgery. Disease relapse Time to relapse
  • 36.  Histological grade is of no significance as ALLEWING’S SARCOMAS ARE CONSIDERED TO BEHIGH GRADE. Fever, anemia, WBC, ESR, LDH indicate extensivedisease so worse prognosis. Aberrant p53 expression & histological response tochemotherapy.