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Abstract
<p class="first" id="P1">Ewing Sarcoma was first described in 1921 in the Proceedings
of the New
York Pathological Society by an eminent American pathologist from Cornell named
James R. Ewing as a “diffuse endothelioma of bone”. Since this
initial description, more has been discovered regarding Ewing Sarcoma and in the
1980’s both Ewing Sarcoma and peripheral primitive neuroectodermal tumors
due to their similar features and shared identical genetic abnormality were
grouped into a class of cancers entitled Ewing Sarcoma Family of Tumors (ESFT).
Ewing Sarcoma is the second most common pediatric osseous malignancy followed by
osteosarcoma, with highest incidence among 10 to 20-year olds. Ewing Sarcoma is
consistently associated with chromosomal translocation and functional fusion of
the
<i>EWSR1</i> gene to any of several structurally related
transcription factor genes of the E26 transformation-specific (ETS) family.
These tumor-specific molecular rearrangements are useful for primary diagnosis,
may provide prognostic information, and present potential therapeutic targets.
Therefore, ways to rapidly and efficiently detect these defining genomic
alterations is of clinical relevance. Within the past decade liquid biopsies,
including extracellular vesicles (EVs), have emerged as a promising alternative
and/or complimentary approach to standard tumor biopsies. It was recently
reported that fusion mRNAs from tumor-specific chromosome translocations can be
detected in Ewing Sarcoma cell-derived exosomes. Within this review, we overview
the current advances in Ewing Sarcoma and the opportunities and challenges in
exploiting circulating exosomes, primarily small bioactive EVs (30–180
nm), as developing sources of biomarkers for diagnosis and therapeutic response
monitoring in children and young adult patients with ESFT.
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