A case of Ewing’s sarcoma
Dr.Ghaniya KC , Dr.C.Jayakumar
Aims , Kochi
Sixteen years old female child, previously healthy, developmentally normal child, presented with complaints of left heel pain since four months. Pain used to be worse at night.
Child developed swelling and pain after a dancing practise and was not able to bear weight since four months.
No history of fever, cough abdominal pain weight loss.
No significant past history.
1st child of NCM
Antenatal, Natal , Post natal history – uneventful
On examination:
Vitals are stable
PICCLE normal
Local exam Diffuse swelling over the calcaneum region no sinus or pus discharge /no limb length discrepancy .
On palpation, fluctuation and tenderness over calcaneum, distal pulses, palpable sensation are intact
Rest of the systems – within normal limits
Investigation: Routine CBC – normal
Inflammatory markers :
CRP 27 ESR 17
FNAC : showed inflammatory infiltrate.
MRI foot marrow infiltrative lesions of multiple Taral bones calcaneal marrow is almost completely replaced sparing only the sustentacular tali
Bone biopsy :Ewing’s sarcoma
Whole body PET : metabolically active destructive light lesions with soft tissue component seen involving almost entire calcaneum with light lesions, involving rest of the bone and base of all metatarsals of left foot
Metabolically, active shaft of left tibia with bone marrow opacities – disease involvement
Metabolically, active left, external iliac, left in vinyl, left lymph nodes– lymph node metastasis
Metabolically, active lytic lesion involving right iliac bone – metastasis.
CT detected multiple subcentrimetric bilateral pulmonary nodules – ? metastasis
Course: Child was started on VAC (Vincristine, Adriamycin, Cyclophosphamide) / IE (Ifosfamide , Etoposide) Q3 weekly regimen
Ewings Sarcoma :
• Rare, aggressive malignant bone tumor.
• Primarily affects children, adolescents, and young adults (5-20 years old).
• Can occur in bones or soft tissue (extraosseous Ewing sarcoma).
Epidemiology:
• 2nd most common bone tumor in children after osteosarcoma.
• Peak incidence: 10-15 years of age.
• Slight male predominance.
• More common in Caucasians; rare in African and Asian populations.
Common Sites:
• Long bones (femur, tibia, humerus).
• Pelvis.
• Ribs.
• Vertebrae.
• Can occur in any bone or soft tissue.
Clinical Presentation:
• Localized bone pain and swelling (most common).
• Pain often worse at night.
• Palpable mass (in advanced cases).
• Pathological fractures.
• Fever, fatigue, and weight loss in advanced stages.
• Neurological symptoms if spinal involvement.
Diagnosis:
• Imaging:
• X-ray: “Onion skin” periosteal reaction (classic finding).
• MRI: Defines soft tissue involvement.
• CT Scan: Evaluates metastases (lungs, bone).
• PET Scan: Staging and monitoring response to treatment.
• Biopsy: Required for definitive diagnosis.
• Histology: Small, round, blue cells.
• Genetics: Associated with t(11;22) translocation (EWSR1-FLI1 fusion gene).
Staging:
• Localized (confined to bone/soft tissue).
• Metastatic (lungs, bones, bone marrow).
Treatment:
• Multimodal approach:
• Chemotherapy (Neoadjuvant and Adjuvant): Vincristine, doxorubicin, cyclophosphamide, etoposide, ifosfamide.
• Surgery: Tumor resection (limb-sparing if possible).
• Radiotherapy: For inoperable or residual tumors.
• High-dose chemotherapy with stem cell transplant for advanced/metastatic disease.
Prognosis:
• 70-80% 5-year survival (localized disease).
• 15-30% 5-year survival (metastatic disease at diagnosis).
• Prognosis worse with larger tumors, pelvic involvement, and poor chemotherapy response.
Follow-up and Late Effects:
• Regular imaging to detect recurrence.
• Monitor for chemotherapy/radiotherapy-related complications (growth issues, secondary cancers, heart/lung toxicity).
Emerging Therapies:
• Immunotherapy and targeted therapies (focus on EWSR1 fusion proteins)