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Primary Pulmonary Myxoid Sarcoma with EWSR1-CREB1 Fusion

MedGen UID:
1379179
•Concept ID:
C4518372
•
Neoplastic Process
Synonyms: Low-Grade Malignant Myxoid Endobronchial Tumor; Primary Pulmonary Myxoid Sarcoma; Primary Pulmonary Myxoid Sarcoma with EWSR1::CREB1 Fusion; Pulmonary myxoid sarcoma with EWSR1-CREB1 translocation; Pulmonary Myxoid Sarcoma with EWSR1-CREB1 Translocation
SNOMED CT: Pulmonary myxoid sarcoma with EWSR1-CREB1 translocation (734072005)

Definition

A sarcoma that arises from the lung. It is related to a bronchus and is often predominantly endobronchial. It is characterized by the proliferation of round and spindle cells within a myxoid stroma. It is associated with the presence of an EWSR1::CREB1 fusion gene. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary Pulmonary Myxoid Sarcoma with EWSR1-CREB1 Fusion

Recent clinical studies

Etiology

EWSR1 Fusions With CREB Family Transcription Factors Define a Novel Myxoid Mesenchymal Tumor With Predilection for Intracranial Location.
Kao YC, Sung YS, Zhang L, Chen CL, Vaiyapuri S, Rosenblum MK, Antonescu CR
Am J Surg Pathol 2017 Apr;41(4):482-490. doi: 10.1097/PAS.0000000000000788. PMID: 28009602Free PMC Article
Tumors with EWSR1-CREB1 and EWSR1-ATF1 fusions: the current status.
Thway K, Fisher C
Am J Surg Pathol 2012 Jul;36(7):e1-e11. doi: 10.1097/PAS.0b013e31825485c5. PMID: 22510762
Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion: a new tumor entity.
Thway K, Nicholson AG, Lawson K, Gonzalez D, Rice A, Balzer B, Swansbury J, Min T, Thompson L, Adu-Poku K, Campbell A, Fisher C
Am J Surg Pathol 2011 Nov;35(11):1722-32. doi: 10.1097/PAS.0b013e318227e4d2. PMID: 21997693

Diagnosis

Primary Pulmonary Myxoid Sarcoma with EWSR1::ATF1 Fusion: A Case Report.
Nishimura T, Ii T, Inamori O, Konishi E, Yoshida A
Int J Surg Pathol 2023 Feb;31(1):88-91. Epub 2022 Apr 24 doi: 10.1177/10668969221095457. PMID: 35466725
Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion: a case report and review of the literature.
Chen Z, Yang Y, Chen R, Ng CS, Shi H
Diagn Pathol 2020 Feb 10;15(1):15. doi: 10.1186/s13000-020-00930-2. PMID: 32039736Free PMC Article
Primary pulmonary myxoid sarcoma located in interlobar fissure without parenchymal invasion.
Kim S, Song SY, Yun JS, Choi YD, Na KJ
Thorac Cancer 2017 Sep;8(5):535-538. Epub 2017 Jul 19 doi: 10.1111/1759-7714.12469. PMID: 28722216Free PMC Article
Primary Pulmonary Myxoid Sarcoma: A Newly Described Entity-Report of a Case and Review of the Literature.
Prieto-Granada CN, Ganim RB, Zhang L, Antonescu C, Mueller J
Int J Surg Pathol 2017 Sep;25(6):518-525. Epub 2017 Apr 28 doi: 10.1177/1066896917706413. PMID: 28449608

Prognosis

Tumors with EWSR1-CREB1 and EWSR1-ATF1 fusions: the current status.
Thway K, Fisher C
Am J Surg Pathol 2012 Jul;36(7):e1-e11. doi: 10.1097/PAS.0b013e31825485c5. PMID: 22510762
Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion: a new tumor entity.
Thway K, Nicholson AG, Lawson K, Gonzalez D, Rice A, Balzer B, Swansbury J, Min T, Thompson L, Adu-Poku K, Campbell A, Fisher C
Am J Surg Pathol 2011 Nov;35(11):1722-32. doi: 10.1097/PAS.0b013e318227e4d2. PMID: 21997693

Clinical prediction guides

Primary Pulmonary Myxoid Sarcoma with EWSR1::ATF1 Fusion: A Case Report.
Nishimura T, Ii T, Inamori O, Konishi E, Yoshida A
Int J Surg Pathol 2023 Feb;31(1):88-91. Epub 2022 Apr 24 doi: 10.1177/10668969221095457. PMID: 35466725

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