Key result
Surgical excision achieves ~85% disease-free survival in primary pulmonary myxoid sarcoma.
Why the study?
To review the epidemiology, clinical characteristics, imaging, pathology, immunohistochemistry, diagnosis, differential diagnosis, treatment, and prognosis of primary pulmonary myxoid sarcoma with EWSR1::CREB1 fusion to provide reference for diagnosis and treatment.
Primary pulmonary myxoid sarcoma with EWSR1::CREB1 fusion is a rare, low-grade malignant lung tumor primarily treated with surgery.
Answers are grounded in this paper and cite their sources.
Supports surgical excision for primary pulmonary myxoid sarcoma; leaves open prospective validation of outcomes and adjuvant needs.
PURPOSE: This review primarily aims to review the epidemiology, clinical characteristics, imaging, pathology, immunohistochemistry, diagnosis, differential diagnosis, treatment, and prognosis of Primary pulmonary myxoid sarcoma (PPMS) with EWS RNA binding protein 1::cAMP response element binding protein 1 (EWSR1::CREB1) fusion. It provides reference for the diagnosis and treatment of this disease. METHODS: Retrospectively collected the literature about PPMS with EWSR1::CREB1 fusion, its clinical, radiology, histology, molecular characteristics and current treatment strategies were collated and analyzed. This review provides a detailed differential diagnosis of the disease. RESULTS: PPMS is an exceptionally rare, low-grade malignant tumor of the lung. This tumor commonly infiltrates lung tissue and develops within bronchial passages. It is identified by a genetic rearrangement involving the EWSR1 gene and a distinct chromosomal translocation t(2; 22)(q33; q12). Variants include EWSR1::CREB1 fusion and EWS RNA binding protein 1::activating transcription factors (EWSR1::ATF1) fusion. PPMS with EWSR1::CREB1 fusion is more prevalent among middle-aged individuals and affects both sexes almost equally. Clinical symptoms are relatively non-specific, primarily including cough, hemoptysis, and weight loss. Most patients undergo surgery and experience a favorable prognosis. Further research is required to validate the effectiveness of alternative treatments for PPMS with EWSR1::CREB1 fusion. CONCLUSION: EWSR1 rearrangement and EWSR1::CREB1 fusion are crucial genetic features of PPMS and serve as important diagnostic markers. Immunohistochemically, PPMS tests positive for EMA. In terms of treatment, surgery has been the primary approach in recent years. Therefore, the efficacy of other treatments still requires further investigation.
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Miao et al. (2024) conducted a review in Primary pulmonary myxoid sarcoma (PPMS) (n=39). Surgery was evaluated on Survival with no evidence of disease (NED). Surgical excision is the primary treatment for primary pulmonary myxoid sarcoma, resulting in 85.3% of surgically treated patients surviving with no evidence of disease at follow-up.
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