Ewing Sarcoma a Dangerous Pitfall for Poorly Differentiated Neuroendocrine Carcinoma (PDEC) in Ileum

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Introduction: Ewing sarcoma (ES) primary to the ileum (IES) has rarely been documented, and shows close similarities in histology and clinical presentation with PDEC.EWSR1-FEV translocation is exceedingly rare in ES, as FEV expression is restricted to prostate, brain, and serotonin neuroendocrine cells (NE) and related tumors.

Aim(s): Little is known about the pathogenesis and clinical implications of IES, which may be critical to identifying novel molecular markers.

Materials and methods: Among 445 ES cases, spanning a period of 20 years, seven (1.6%) were IES. Seven IES were investigated through immunohistochemistry, RT-PCR (EWSR1-FLI1,EWSR1-ERG and EWSR1-FEV transcripts), FISH analysis (EWSR1 break-apart and specific EWSR1-FEV translocation) and spectral karyotyping (SKY). Ten ileum neuroendocrine tumors (INET) made up the control group for EWSR1-FEV translocation.

Conference: 11th Annual ENETSConcerence (2014)

Presenting Author: Milione M

Authors: Milione M, Gasparini P, Coppa J, Pusceddu S, Pellegrinelli A,

Keywords: ileum, neuroendocrine, Ewing Sarcoma, EWSR1,