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The final flask contained a mutant strain of yeast lacking ATF1
. It lured zero flies.
(40) Occasional cases with EWSR1 rearrangement have failed to show involvement of ATF1 or CREB1, suggesting that these may have as yet unidentified fusion partners.
Fusion of the EWSR1 and ATF1 genes without expression of the MITF-M transcript in angiomatoid fibrous histiocytoma.
Fusion of the FUS and ATF1 genes in a large, deep-seated angiomatoid fibrous histiocytoma.
gene, located at 12q13.12, encodes activating transcription factor 1 (ATF1
) protein, which belongs to the ATF subfamily and basic-region leucine zipper (bZIP) family.
EWSR1 (Vysis LSI EWSR1 22q12 dual-color break-apart probe, Abbott Molecular, Abbott Park, Illinois) and ATF1 (custom break-apart probe) probes were used for hybridization.
A recent series described tumors with features similar to CCS-like gastrointestinal tumor as "malignant gastrointestinal neuroectodermal tumor." (15) These tumors of the GI tract share morphologic, immunohistochemical, and molecular similarities with our case, such as the presence of EWSR1 rearrangement in most cases, with 46% showing ATF1 and 22% showing CREB1 rearrangement.
While EWSR1-ATF1 translocation may not entirely define a tumor as CCS (eg, this translocation is also seen in angiomatoid fibrous histiocytoma), the presence of EWSR1 and ATF1 rearrangements, in conjunction with the morphologic and immunohistochemical features seen in our case, supports a diagnosis of CCS.
This translocation results in fusion of the 3' portion of the Ewing sarcoma (EWSR1) oncogene on chromosome 22q with the 3' portion of the activating transcription factor 1 (ATF1
) oncogene on chromosome 12q, giving rise to the EWSR1/ATF1 chimeric transcript.