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Primary pulmonary myxoid sarcoma located in interlobar fissure without parenchymal invasion
Seok Kim1, Sang Yun Song1, Ju Sik Yun1
1Department of Thoracic and Cardiovascular Surgery, Chonnam National University Hwasun Hospital, Chonnam National University School of Medicine, Jeollanam-do, South Korea.
Abstract:
Primary pulmonary myxoid sarcoma (PPMS), classified as low to intermediate grade malignant myxoid endobronchial tumor, is rarely reported. Most reported cases occurred in lung parenchyme with an endobronchial component. Herein, we report a case of PPMS in a 29-year-old woman that developed in a major fissure of the left lung without parenchymal invasion. Histopathologically, the diagnosis was compatible to PPMS with EWSR1-CREB1 translocation.
Insights
Primary pulmonary myxoid sarcoma (PPMS) is a rare, low to intermediate grade malignant tumor. This report details a unique case of PPMS in a major lung fissure, confirmed by EWSR1-CREB1 translocation.
Area of Science:
- Pulmonology
- Oncology
- Pathology
Background:
- Primary pulmonary myxoid sarcoma (PPMS) is a rare malignant neoplasm.
- It is typically classified as a low to intermediate grade myxoid endobronchial tumor.
- Most reported cases involve lung parenchyma with an endobronchial component.
Observation:
- This study presents a rare case of PPMS.
- The tumor occurred in a 29-year-old woman.
- It developed within a major fissure of the left lung without parenchymal invasion.
Findings:
- Histopathological examination confirmed the diagnosis of PPMS.
- The specific genetic hallmark, EWSR1-CREB1 translocation, was identified.
- This case represents an unusual presentation of PPMS.
Implications:
- This case expands the understanding of PPMS presentation.
- It highlights the importance of considering rare diagnoses in pulmonary oncology.
- Further research into PPMS genetics and behavior is warranted.

