Published on March 5, 2014
EWING’S SARCOMA PRITHWIRAJ MAITI FINAL YEAR MBBS R.G.KAR MEDICAL COLLEGE 5.3.2014
Introduction • It is the 3rd most common primary malignant bone tumor. • Arises from endothelial cells of bone marrow. • Age: First 2 decades of life. • Common in males. • Uncommon in blacks.
• Location affected: Diaphysis of long bone. • Common bones involved: (in decreasing order) 1. Femur, 2. Tibia, 3. Fibula, 4. Humerus, 5. Flat bones.
Clinical features • • • • • • Throbbing, intermittent pain. Pain worst at night. Red and warm skin. Swelling over diaphyseal region. Generalized illness and pyrexia. Mimics acute osteomyelitis.
Pathology • Lobulated large mass over diaphysis of long bone. • When hemorrhage occurs, it appears like a red current jelly. • Extensive medulary invasion and destruction of endosteum and cortex. • Prominent peiosteal new bone formation. • Sometimes, the tumor is liquefied so as to resemble a pus.
Histology • • • • • • Highly cellular. Little intercellular matrix. Necrosis is common. Cells are arranged around the vessels. PAS +Ve (due to presence of glycogen). HBA71 +Ve (it is an immunological marker).
Investigations • Blood: Anemia, Leucocytosis, Raised ESR, Raised LDH. • X-Ray: Diaphyseal lesion with irregular destruction (Moth eaten appearance). Periosteal new bone formation (Onion peel appearance). X-Ray chest (to detect metastasis).
• CT Scan: Detect the extent of cortical destruction. CT scan chest: Detection of metastasis. • MRI: Detection of the degree of intraosseus and extraosseus extension of tumor. Initial staging and surgical planning. Assessment of response of the tumor to chemotherapeutic regimen.
• Bone scan: Detection of the extent of tumour involvement. Detection of the skip lesions in the same bone. Detection of distant metastasis.
Course • Repeated episodes of exacerbation and remission is characteristic. • Metastasize by hematogenous and lymphatogenous routes. • It obeys the course of bone to bone metastasis (another one example is osteosarcoma). • Common bones where Ewing’s sarcoma metastasizes are skull, vertebra, rib and lung.
Management of Ewing’s sarcoma 1. Radiotherapy, 2. Surgery, 3. Chemotherapy.
Radiotherapy • Ewing’s sarcoma is highly radiosensitive tumour. • Radiotherapy has dramatic effect on Ewing’s sarcoma (Melts like snow). • But recurrence rates are high in case of radiotherapy alone, survival rates are also not satisfactory.
Surgery • Surgery has a very little role in the management of Ewing sarcoma. • The available options are: 1. Debulking surgery. 2. Limb preservation surgery.
Systemic Chemotherapy • It is the mainstay of treatment of Ewing’s sarcoma. • Response to chemotherapy is the most important prognostic factor in this disease. • Common agents used are: Ifosfamide/ Actinomycin D/ Doxorubicin/ Vincristine etc.
Comment Best result can be achieved only by combining all the 3 options available.
Prognostic Factors • • • • • • • • Site: Humerus and pelvis-> Bad prognosis. Stage: Metastasis-> Bad prognosis. Tumour size. Response to chemotherapy. Male gender. High LDH level. Anemia. c-MIC/ ki-67 gene expression.
Ewing's sarcoma is more common in males (1.6 male:1 female) and usually presents in childhood or early adulthood, with a peak between 10 and 20 years of age.
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nach dem US-amerikanischen Pathologen James Ewing (1866-1943) Englisch: Ewing's sarcoma