Primary pulmonary synovial sarcoma presenting with a mass in the right upper mediastinum: A case report

  • Cung Văn Công Bệnh viện Phổi Trung ương

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Keywords

Soft tissue sarcoma, synovial sarcoma, primary pleural synovial sarcoma

Abstract

Synovial sarcoma (SS) represents a unique subset of soft tissue sarcomas (STS) and accounts for 5–10% of total STS. Primary Pulmonary Synovial Sarcoma (PPSS) is an extremely rare entity, especially in children and adolescents. Because of the rarity of this site and the generation of histopathological changes, the diagnosis of PPSS has always been a clinical challenge.      Similar to other STS, the diagnosis of SS is often a combination of history, physical examination, magnetic resonance imaging, computed tomography-guided biopsy, histopathology, immunohistochemistry, and molecular biology. Extensive resection of the tumor remains the standard for definitive treatment of the disease, with adjuvant radiation used for larger lesions. There is still much controversy surrounding the role of chemotherapy in the treatment of SS. Today, as the understanding of the molecular and immunological features of SS develops, several potential systemic therapies have been proposed.        We introduce a rare case of PPSS to the National Lung Hospital for examination and treatment with a mediastinal tumor presenting all the diagnostic and treatment criteria for colleagues' reference.

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References

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