EWSR1 and ATF1 rearrangements in clear cell odontogenic carcinoma: presentation of a case.

Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor of the jaws that is more common in the mandible than maxilla and has a female preponderance with a peak incidence in the sixth decade. It is characterized by locally aggressive behavior and has the potential to metastasize. This tumor was recently reported to have a rearrangement of the Ewing sarcoma breakpoint region 1 gene (EWS RNA-binding protein 1, EWSR1) in 5 of 8 cases tested and of the activating transcription factor 1 gene (ATF1) in 1 case tested. We report a case of CCOC in the premolar area of the mandible in a 59-year-old woman. This case demonstrated the presence of both EWSR1 and ATF1 gene rearrangements by fluorescence in situ hybridization.
AuthorsAaron E Yancoskie, Chandrika Sreekantaiah, Jessy Jacob, Amanda Rosenberg, Morris Edelman, Cristina R Antonescu, John E Fantasia
JournalOral surgery, oral medicine, oral pathology and oral radiology (Oral Surg Oral Med Oral Pathol Oral Radiol) Vol. 118 Issue 4 Pg. e115-8 (Oct 2014) ISSN: 2212-4411 [Electronic] United States
PMID24721473 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2014 Elsevier Inc. All rights reserved.
Chemical References
  • Calmodulin-Binding Proteins
  • EWSR1 protein, human
  • RNA-Binding Proteins
  • Adenocarcinoma, Clear Cell (diagnosis, genetics, pathology, surgery)
  • Biopsy
  • Calmodulin-Binding Proteins (genetics)
  • Cone-Beam Computed Tomography
  • Diagnosis, Differential
  • Diagnostic Imaging
  • Female
  • Gene Rearrangement
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Mandibular Neoplasms (diagnosis, genetics, pathology, surgery)
  • Middle Aged
  • Odontogenic Tumors (diagnosis, genetics, pathology, surgery)
  • RNA-Binding Proteins (genetics)

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