Primary palatal sarcoma exhibiting EWSR1::RORß fusion: a first case report and literature review

Haein Park; Daniel Wilfredo Banegas; Seung Yong Han; Hyun Sil Kim; In Ho Cha; Hyang Joo Ryu; Dongwook Kim

doi:10.1016/j.oooo.2024.06.018

Primary palatal sarcoma exhibiting EWSR1::RORß fusion: a first case report and literature review

Haein Park, Daniel Wilfredo Banegas, Seung Yong Han, Hyun Sil Kim, In Ho Cha, Hyang Joo Ryu, Dongwook Kim

Research output: Contribution to journal › Article › peer-review

Abstract

In this report, a tumor exhibited EWSR1::RORß gene fusion, to our knowledge, is the first such reported case. The Ewing sarcoma breakpoint region 1 gene (EWSR1) is known to be associated with several soft tissue tumors although its specific role remains unclear. Its fusion with a member of the ETS family, including FLI1 and ERG, results in Ewing sarcoma, and its fusion with other genes unrelated to the ETS family, including NFATC2 and PATZ1, results in round cell sarcoma with EWSR1-non-ETS fusions, previously referred to as Ewing-like sarcoma. RORß encodes retinoic acid-related orphan receptor ß, a nuclear receptor (NR), and is involved in circadian rhythm modulation and cancer regulation. The specific role of RORß in tumorigenesis remains unclear; however, this case report suggests that it may form part of a new tumorigenic entity.

Original language	English
Pages (from-to)	e113-e119
Journal	Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Volume	138
Issue number	6
DOIs	https://doi.org/10.1016/j.oooo.2024.06.018
Publication status	Published - 2024 Dec

Bibliographical note

Publisher Copyright:
© 2024 Elsevier Inc.

All Science Journal Classification (ASJC) codes

Surgery
Oral Surgery
Pathology and Forensic Medicine
Dentistry (miscellaneous)
Radiology Nuclear Medicine and imaging

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1016/j.oooo.2024.06.018

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title = "Primary palatal sarcoma exhibiting EWSR1::ROR{\ss} fusion: a first case report and literature review",

abstract = "In this report, a tumor exhibited EWSR1::ROR{\ss} gene fusion, to our knowledge, is the first such reported case. The Ewing sarcoma breakpoint region 1 gene (EWSR1) is known to be associated with several soft tissue tumors although its specific role remains unclear. Its fusion with a member of the ETS family, including FLI1 and ERG, results in Ewing sarcoma, and its fusion with other genes unrelated to the ETS family, including NFATC2 and PATZ1, results in round cell sarcoma with EWSR1-non-ETS fusions, previously referred to as Ewing-like sarcoma. ROR{\ss} encodes retinoic acid-related orphan receptor {\ss}, a nuclear receptor (NR), and is involved in circadian rhythm modulation and cancer regulation. The specific role of ROR{\ss} in tumorigenesis remains unclear; however, this case report suggests that it may form part of a new tumorigenic entity.",

author = "Haein Park and Banegas, {Daniel Wilfredo} and Han, {Seung Yong} and Kim, {Hyun Sil} and Cha, {In Ho} and Ryu, {Hyang Joo} and Dongwook Kim",

note = "Publisher Copyright: {\textcopyright} 2024 Elsevier Inc.",

year = "2024",

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doi = "10.1016/j.oooo.2024.06.018",

language = "English",

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T1 - Primary palatal sarcoma exhibiting EWSR1::RORß fusion: a first case report and literature review

AU - Park, Haein

AU - Banegas, Daniel Wilfredo

AU - Han, Seung Yong

AU - Kim, Hyun Sil

AU - Cha, In Ho

AU - Ryu, Hyang Joo

AU - Kim, Dongwook

PY - 2024/12

Y1 - 2024/12

N2 - In this report, a tumor exhibited EWSR1::RORß gene fusion, to our knowledge, is the first such reported case. The Ewing sarcoma breakpoint region 1 gene (EWSR1) is known to be associated with several soft tissue tumors although its specific role remains unclear. Its fusion with a member of the ETS family, including FLI1 and ERG, results in Ewing sarcoma, and its fusion with other genes unrelated to the ETS family, including NFATC2 and PATZ1, results in round cell sarcoma with EWSR1-non-ETS fusions, previously referred to as Ewing-like sarcoma. RORß encodes retinoic acid-related orphan receptor ß, a nuclear receptor (NR), and is involved in circadian rhythm modulation and cancer regulation. The specific role of RORß in tumorigenesis remains unclear; however, this case report suggests that it may form part of a new tumorigenic entity.

AB - In this report, a tumor exhibited EWSR1::RORß gene fusion, to our knowledge, is the first such reported case. The Ewing sarcoma breakpoint region 1 gene (EWSR1) is known to be associated with several soft tissue tumors although its specific role remains unclear. Its fusion with a member of the ETS family, including FLI1 and ERG, results in Ewing sarcoma, and its fusion with other genes unrelated to the ETS family, including NFATC2 and PATZ1, results in round cell sarcoma with EWSR1-non-ETS fusions, previously referred to as Ewing-like sarcoma. RORß encodes retinoic acid-related orphan receptor ß, a nuclear receptor (NR), and is involved in circadian rhythm modulation and cancer regulation. The specific role of RORß in tumorigenesis remains unclear; however, this case report suggests that it may form part of a new tumorigenic entity.

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Primary palatal sarcoma exhibiting EWSR1::RORß fusion: a first case report and literature review

Abstract

Bibliographical note

All Science Journal Classification (ASJC) codes

UN SDGs

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