Ewing's Sarcoma Family of Tumors: Current Management

Abstract

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NCCN Guidelines Insights: Bone Cancer, Version 2.2017

“…3,4 With current multimodality treatment, approximately threequarters of all patients diagnosed with osteosarcoma are cured, and 90% to 95% of patients diagnosed with osteosarcoma can be successfully treated with limb-sparing approaches rather than amputation. 5 Survival rates have improved to almost 70% in patients with localized Ewing sarcoma.…”

A Rare Case of Ewing’s Sarcoma affecting Mandible of a Child

“…Ewing's sarcoma is a malignantsmall round cell tumor of primitive neuro ectodermal tumor group and accounts for around 5% all bone malignancy 2 .It shows a characteristic translocation t(11:22) (q24:q12) resulting in the fusion of the EWS and FLI-I genes. This gene rearrangement causes a fusion product which functions as an oncogenic aberrant transcription factor with structural variability 5 .…”

“…Usually it affects the long bone & in the maxillofacial region mandible is the longest bone,it involves the mandible mostly.Less than 3% of all ES originate in the maxillofacial region, usually involving the mandible 1,2 . 90% occur in the first three decades of life and males are more often affected than females with the ratio of 3:2 1,3 .…”

Expression of ADAMTS4 in Ewing's sarcoma

“…Currently, diagnosis of EWS is determined mainly by CD99 immunohistochemistry (11)(12)(13), and by genetic aberration. However, CD99 expression is also reported to be positive in some T cell acute lymphoblastic leukemia (T-ALL), acute myelogenous leukemia (AML), ependymoma, synovial sarcoma and pancreatic endocrine tumors (14)(15)(16).…”