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Journal Of Modern Oncology[ISSN:1672-4992/CN:61-1415/R]

Issue:

2024 22

Page:

4311-4316

Research Field:

Publishing date:

Info

Title:

Primary pulmonary myxoid sarcoma with EWSR1-CREB1 gene fusion:A clinicopathological analysis

Author(s):

ZHANG Yunrong¹; PAN Taoqiang¹; CAO Wei¹; MA Jun¹; ZHANG Dawei²; YU Zhaoxia¹

1.Department of Pathology;2.Department of Radiology,Anqing First People's Hospital,Anhui Anqing 246000,China.

Keywords:

primary pulmonary myxoid sarcoma; low-grade sarcoma; clinical pathology; EWSR1-CREB1 fusion

PACS:

R734.2

DOI:

10.3969/j.issn.1672-4992.2024.22.015

Abstract:

Objective:To investigate the clinicopathologic characteristics,immunohistochemical and genetic features of primary pulmonary myxoid sarcoma(PPMS) with EWSR1-CREB1 gene fusion.Methods:Analyze the clinical and pathological characteristics of a case of PPMS with EWSR1-CREB1 gene fusion,and review relevant literature.Results:The tumor was closely related to the bronchus,appearing in a lobulated shape.The tumor cells appear as solid patchy and reticular distribution in a mucinous background.The cells were oval or circular in shape,with occasional mitotic figures and no necrosis observed.There was interstitial infiltration with lymphocytes and plasma cells.Immunophenotypes showed vimentin (+),SMA (focal+),Ki-67 (10%+) and TTF-1,EMA,p63,Desmin,HMB45,Melan-A,S-100,CD34,¦Â-catenin and CD99 were all negative.Molecular detection:FISH and NGS detected EWSR1-CREB1 fusion.After 15 months of follow-up after surgical treatment,the patient survived with disease-free.Conclusion:PPMS is a rare primary low-grade sarcoma of the lung with no specific morphological and immunophenotypic features.It requires comprehensive diagnosis by combining FISH,RT-PCR,or NGS detection.

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