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Review
. 2020 Nov 23;13(11):e237678.
doi: 10.1136/bcr-2020-237678.

Primary synovial sarcoma of the lung: a rare presentation, diagnostic dilemma and review of literature

Laxman Pandey  1 , Deepa Joseph  1 , Rajesh Pasricha  2 , Manoj Kumar Gupta  1
Affiliations
Review

Primary synovial sarcoma of the lung: a rare presentation, diagnostic dilemma and review of literature

Laxman Pandey et al. BMJ Case Rep. .

Abstract

Primary synovial sarcoma of the lung (PSSL) is a rare tumour with only 0.5% incidence among other primary malignant lung tumours. Published medical literature regarding the natural history treatment protocol and clinical outcomes of PSSL remains limited. Here, we present a case of a 39-year-old man with primary synovial sarcoma with long-term follow-up, which was initially viewed as a metastasis from another unknown site. After evaluating histopathologically from the specimen post left lung metastasectomy and ruling out any other site for neoplastic changes, the diagnosis of PSSL was confirmed. Furthermore, this article reviews the literature and discusses various aspects of PSSL, including clinical presentation, radiological imaging and pathological characteristics, diagnostic dilemma, management and prognosis. This paper serves to provide an update and aims to enhance the understanding of PSSL. Timely diagnosis and treatment are imperative to achieve improved outcomes.

Keywords: radiotherapy; respiratory cancer.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Positron emission tomography (PET)-CT showing soft tissue heterogamous mass lesion in the lingular segment of the left lung (3.5 cm × 3.7 cm) of standardised uptake value (SUV) max 3.7.
Figure 2
Figure 2
(A) A highly cellular tumour showing circumscription. Uninvolved lung parenchyma is identified in the lower left corner. (B) Higher magnification of the tumour (200 ×, H&E stain) shows sheets of tumour cells having high N:C ratio. (C) Strong staining with TLE-1. (D) Tumour cells also showing moderate intensity of staining with CD 99. (E) Peripheral part of the tumour shows staining with pancytokeratin. (F) Peripheral part of the tumour shows staining with epithelial membrane antigen (EMA). (G) Diffuse cytoplasmic staining is seen with BCL-2. (H) No staining seen with CD34. (I) ki-67 labelling index in the highest proliferating areas is 20%–25%.
Figure 3
Figure 3
Cell showing one green, one orange and one green-orange (fusion) signal indicating positive for SYT gene rearrangement.
See this image and copyright information in PMC

References

    1. Frazier AA, Franks TJ, Pugatch RD, et al. . From the Archives of the AFIP: pleuropulmonary synovial sarcoma. Radiographics 2006;26:923–40. 10.1148/rg.263055211 - DOI - PubMed
    1. Keel SB, Bacha E, Mark EJ, et al. . Primary pulmonary sarcoma: a clinicopathologic study of 26 cases. Mod Pathol 1999;12:1124–31. - PubMed
    1. Murphey MD, Gibson MS, Jennings BT, et al. . From the Archives of the AFIP: imaging of synovial sarcoma with radiologic-pathologic correlation. Radiographics 2006;26:1543–65. 10.1148/rg.265065084 - DOI - PubMed
    1. Singhal S, Prajapat DK, Sharma RK, et al. . Primary pulmonary synovial sarcoma: is it worth all the hard work? J Assoc Chest Physicians 2019;7:29–34. 10.4103/jacp.jacp_15_18 - DOI
    1. Zeren H, Moran CA, Suster S, et al. . Primary pulmonary sarcomas with features of monophasic synovial sarcoma: a clinicopathological, immunohistochemical, and ultrastructural study of 25 cases. Hum Pathol 1995;26:474–80. 10.1016/0046-8177(95)90242-2 - DOI - PubMed