Statement of Clinical Relevance
- •ECT is an uncommon tumor with a wide spectrum of histopathologic and immunohistochemical features. A thorough review of its characteristics is beneficial to the treating clinicians and pathologists as they diagnose and manage patients with this rare neoplasm.
Abstract
Objective
: Ectomesenchymal chondromyxoid tumor (ECT) is an uncommon soft tissue tumor with an
enigmatic histogenesis and striking predilection for the tongue. We present 5 new
cases and review the literature.
Study Design
: We performed a retrospective search for ECTs within the University of Kentucky Oral
Pathology Biopsy Service and the published literature.
Results
: Five new cases from the biopsy archives and 103 well-documented ECT cases from the
literature were compiled and reviewed. 89.8% of ECT are found on the anterior/dorsal/lateral/unspecified
tongue, while 4.6% are on the posterior/base of tongue. Six extralingual cases are
reported. The age ranges from 2.3-78 years with an average of 40. Most ECT react with
GFAP (92.8%) and S-100 protein (91.3%). 21/23 cases demonstrate a RREB1::MKL2 fusion, while EWSR1 gene mutations are identified in 4 cases.
Conclusions
: Most ECT are readily diagnosed on routine histopathology in combination with tumor
site, immunohistochemical findings, and molecular findings; however, a subset share
overlapping features with myoepithelioma of soft parts. As further molecular analysis
is performed on this tumor, we may find that a subset of previously diagnosed ECT
relate to or represent myoepithelioma or conversely fall under the spectrum of the
pluripotent ECT.
Keywords
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Article info
Publication history
Accepted:
December 13,
2022
Received in revised form:
November 2,
2022
Received:
September 27,
2022
Publication stage
In Press Journal Pre-ProofIdentification
Copyright
© 2022 Elsevier Inc. All rights reserved.
