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Review
. 2024 Mar 25;18(1):25.
doi: 10.1007/s12105-024-01625-6.

A Comparison of Clear Cell Sarcoma to Jaw and Salivary Tumors Bearing EWS Fusions

Ornela Xhori  1 , Navkiran Deol  2   3 , Camron M Rivera  4   1 , Jason Zavras  5 , Sophia G Weil  6 , Hirad Zafari  4 , Julia C Thierauf  7 , William C Faquin  7   8 , Edwin Choy  8   9 , Miguel N Rivera  7   8 , A John Iafrate  7   8 , Alexandre Jaquinet  10 , Maria J Troulis  4   1
Affiliations
Review

A Comparison of Clear Cell Sarcoma to Jaw and Salivary Tumors Bearing EWS Fusions

Ornela Xhori et al. Head Neck Pathol. .

Abstract

Objective: To review tumors identified as "clear cell sarcoma" in order to determine similarities to the rare EWS fusion positive jaw and salivary gland tumors clear cell odontogenic carcinoma (CCOC) and clear cell carcinoma of the salivary gland (CCC).

Methods: PubMed was used to collect all reports of clear cell sarcoma (CCS). Search parameters were "clear cell sarcoma" and "CCS." References in the publications were screened and cross-referenced. Data extracted included demographic characteristics, presenting signs and symptoms, radiographic findings, histological and immunohistochemical features and known molecular/genetic aberrations.

Results: Clear cell sarcoma has several similarities to CCOC and CCC. All three tumor types have similar histologic appearances including the presence of clear cells, as well as similar genetic profiles in that all harbor an EWSR1-CREB family fusions. Additionally, these tumors appear in soft tissue as well as bone, and can have a prolonged clinical course. CCS can appear anywhere in the body, including the head and neck region. All three tumors appear to have a predilection to women, although CCS may have a slight younger age of onset as compared to CCOC and CCC (3rd vs 5th decade of life, respectively).

Conclusion: Gaining a better understanding of the similarities and differences between these three tumors may lead to a better understanding of each one.

Keywords: ATF1; CREB1; CREM; Clear cell carcinoma of the salivary gland; Clear cell odontogenic carcinoma; Clear cell sarcoma; Clear cells; EWSR1.

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Conflict of interest statement

The authors have no competing interests to declare that are relevant to the content of this article.

Figures

Fig. 1
Fig. 1
CCS demographics of 455 reported cases
Fig. 2
Fig. 2
Distribution of CCS Subtypes in 501 Cases
Fig. 3
Fig. 3
Distribution of 30 CCS Cases in the Head and Neck Region
Fig. 4
Fig. 4
a CCS occurrence predominantly in the lower extremity of the body. b Breakdown of lower extremity CCS cases, showing the highest frequency in the foot. c Distribution of CCS cases across the upper extremity, with the hand being the most common site. d Central body locations of CCS with the thorax as the most frequent site
Fig. 5
Fig. 5
Clinical presentations of 227 cases of CCS
Fig. 6
Fig. 6
Distribution of imaging modalities used in the evaluation of 137 CCS cases
See this image and copyright information in PMC

References

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