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

© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature..

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2024

Erschienen:

2024

Enthalten in:

Zur Gesamtaufnahme - volume:18

Enthalten in:

Head and neck pathology - 18(2024), 1 vom: 25. März, Seite 25

Sprache:

Englisch

Beteiligte Personen:

Xhori, Ornela [VerfasserIn]
Deol, Navkiran [VerfasserIn]
Rivera, Camron M [VerfasserIn]
Zavras, Jason [VerfasserIn]
Weil, Sophia G [VerfasserIn]
Zafari, Hirad [VerfasserIn]
Thierauf, Julia C [VerfasserIn]
Faquin, William C [VerfasserIn]
Choy, Edwin [VerfasserIn]
Rivera, Miguel N [VerfasserIn]
John Iafrate, A [VerfasserIn]
Jaquinet, Alexandre [VerfasserIn]
Troulis, Maria J [VerfasserIn]

Links:

Volltext

Themen:

ATF1
CREB1
CREM
Clear cell carcinoma of the salivary gland
Clear cell odontogenic carcinoma
Clear cell sarcoma
Clear cells
EWSR1
Journal Article
Oncogene Proteins, Fusion
RNA-Binding Protein EWS
Review

Anmerkungen:

Date Completed 27.03.2024

Date Revised 28.03.2024

published: Electronic

Citation Status MEDLINE

doi:

10.1007/s12105-024-01625-6

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM370163370