Ewing’s sarcoma

 Symptoms |Signs |Treatment

 

Ewing’s sarcoma is a round cell tumor, a component of the Ewing’s Sarcoma Family Of Tumours (EFTS).

What is the Incidence ?

  • It is rare cancer
  • But the second most common primary bone cancer.

    Pelvic Ewings
    Ewing’s Sarcoma of pelvis in Pediatric

What ages does it affect ?

  • Seen in the age group of 5-20 years commonly.
  • Adults are uncommonly affected.

What is the cause ?

  • The etiology of Ewing’s sarcoma is evident as reciprocal translocation between chromosome 11 and 22 in 85 % of cases.
  • EWSR1 and FLI1 fusion are identified in the majority of cases on Rt-PCR & FISH.
  • Immunohistochemistry (IHC) identifies CD99, Fli-1 as confirmatory.

Which bones does it affect ?

  • Long bones
  • Pelvis
  • Chest wall
  • Spine, clavicle, hand and foot
  • Extraosseous (Soft tissue) commonly seen in trunk and extremity

How is Ewing’s Sarcoma identified?

Ewing’s sarcoma | Symptoms | Signs | Treatment
  • Symptoms
    • Pain (Night Pain, Rest Pain)
    • Swelling (Rapidly progressive)
    • Masquerades as infection of bone in some instances with purulent discharge, may have fever as main symptom
    • Pathological fracture due to trivial trauma
    • In advanced cases, loss of weight , appetite

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  • Tests
    • Plain Radiograph
    • MRI with contrast
    • CT scan
    • Image guided biopsy
    • Whole body FDG PET CT scan
    • Blood tests-LDH, ALP, ESR, CBC
  • Nearly 75 % of individuals present with a solitary primary tumor without any distant metastases.
  • Around 10 % of patients present with microscopically detectable bone metastases.

What tests are done on biopsy tissue?

  • Histopathological evaluation
  • Cytogenetic analysis
  • Immunohistochemistry
  • Flow Cytometry

What are the treatment Options ?

  • The treatment of Ewing’s sarcoma is by a multidisciplinary team similar to osteosarcoma.
  • Chemotherapy (Various regimens are used, 2 weekly interval Dose intense regimen is preferable)
    • Preoperative
    • Evaluation of Chemotherapy response by Imaging & Evaluation of specimen
    • Postoperative
  • Surgery: Limb salvage in 95% of cases.
  • Type of Limb salvage surgery will be individualized.
  • Rarely, amputation

  • Radiotherapy: Effective is Ewing’s sarcoma.
  • Often given as Post operative (Often based on size, operative margin)
  • Rarely, as preoperative along with chemotherapy to shrink the tumour.
  • Rarely, instead of surgery (Reserved for Non operable or High risk cases in pelvis, spine, sacrum and large size of primary tumor. Risk of recurrence is 10-20 %).
  • High dose chemotherapy with stem cell rescue
  • Targeted therapy, Immunotherapy

How is Limb Salvage possible in Ewing’s Sarcoma?

Ewing's sarcoma Limb salvage
Limb salvage surgery for Ewing’s sarcoma Tumor Megaprosthesis
  • Certain bones in our body are expendable and do not require any further reconstructive or salvage procedures like clavicle, rib, scapula, sternum, distal ulna and ilium.
  • The cancer is removed En-Bloc with wide margins
  • Reconstruction options include the same modalities as discussed in osteosarcoma which are individualized.
    • These are Bone Grafts (Autologous or Allogeneic) which can be segmental (intercalary) or osteoarticular aided with implants
    • Metallic Endoprosthesis (Megaprosthesis)
    • Allograft-Prosthesis Composite (APC)
    • Extracorporeal radiation and Re-implantation (ECRT)
    • Vascularized fibula construct
    • Allograft resurfacing.
    • In children and adolescents who have tumors involving growth plate and residual growth remaining, an APC or Expandable Endoprosthesis (invasive or non-invasive) are performed.
    • In individuals who have attained skeletal maturity, most common options are adult endoprosthesis and reconstruction using different modes of Bone grafts as outlined above.
    • In case of involvement of shaft or diaphysis of long bones and pelvis, the removed tumor bone can be irradiated and reimplanted or substituted with allograft.
    • Rehabilitation is provided according to the type of reconstruction.

What is the treatment for Metastatic disease?

  • Most common site is Lung. Bone involvement is uncommon.
  • A whole body fdg pet ct scan follows for staging.
  • If an individual has distant metastases (lung most common), it can be surgically removed after removal of the primary tumor.
  • If lung involvement is extensive then Radiotherapy to lung is provided
  • Chemotherapy is necessary, but regimens of drugs may differ.

What are the rehabilitation steps involved post limb salvage?

  • Rehabilitation post-surgery is dependent on the type of limb salvage.
  • In case of bone graft reconstruction or APC, the involved limb is protected from bearing load or weight for a duration of 6 – 9 months to allow sufficient healing and bone graft incorporation.
  • When endoprosthesis reconstruction is performed in pelvis and lower limbs, protected weight bearing and walking is allowed from the second postoperative day.

How often should I visit the doctor?

  • The individual is followed up every 3 months for the first 2 years, followed by 6 months every 3 years (aggressive monitoring of the patient until 5 years).
  • Overall survival of primary non metastatic Ewing’s sarcoma at presentation is 60 – 70 %.
  • Distant metastases and local recurrence may necessitate change in chemotherapy protocol depending on the nature of event.
  • Solitary metastasis and local recurrences require to be surgically removed.
  • Survival of this specific category of individuals is 20-30 % at 5 years.
  • Occurrence of multiple widespread metastases or multiple local recurrences increase morbidity and mortality risk.

Are there any prognostic factors for Ewing’s sarcoma?

  • Stage of disease

Better prognosis is for primary tumor without metastasis

  • Bone or Soft tissue involvement
  • long bone involvement compared to pelvis and axial sites,
  • small tumor size (less than 200 ml)
  • children compared to adults
  • lung metastases compared to bone metastasis
  • response to initial chemotherapy surgery and radiotherapy
  • Combination of Surgery and Radiotherapy
  • LDH, Alkaline Phosphatase levels
  • Gene changes
  • Pathological Fracture
  • In case of recurrence, the site and stage

 

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    Apollo Hospitals, Bengaluru
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