{"paper_id":"44b0d742-bf98-4909-abe0-4e098701e7f3","body_text":"An Unusual Case of Primary Pulmonary Synovial Sarcoma | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report An Unusual Case of Primary Pulmonary Synovial Sarcoma SACHINKUMAR DOLE, M S BARTHWAL This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-5206970/v1 This work is licensed under a CC BY 4.0 License Status: Under Review Version 1 posted 2 You are reading this latest preprint version Abstract Background : An extremely rare intrathoracic tumour that typically affects young adults is primary pulmonary synovial sarcoma. It manifests as a big intrathoracic mass without involvement of the bone or mediastinal region. Tumour biopsy, immunostaining and histologic analysis can assist in determining the kind of tumour. Case Presentation : We present a case of a 25 year old male with complaints of breathlessness and chest pain for a month. On radiological imaging he was found to have mass in upper and middle zones of right hemithorax. For confirmation of diagnosis patient underwent ultrasound guided biopsy of the lesion and histopathologhical examination (HPE) of the lesion. The histopathological features were suggestive of a rare type of lung malignancy. For further diagnosis regarding type of malignancy, immunohistochemical staining of the lesion was done with the help of with specific immunological markers, which confirmed the diagnosis of monophasic pulmonary sarcoma. On PET (positron emission tomography) CT (computed tomography) scan there were no features suggestive of distant metastasis especially in extremities which confirmed the diagnosis of primary pulmonary synovial sarcoma. Patient was started on chemotherapy regimen of Ifosfamide and Doxorubicin after oncologist’s opinion but was lost to follow up after 3 rd cycle of chemotherapy. Conclusions : Primary pulmonary synovial sarcoma is an extremely uncommon tumor in young adults as lung metastases from other sources mainly extremities are more common. Diagnosis of such rare tumour requires histopathological examination along with immunohistochemical staining of the lung lesions. A multimodal strategy of treatment involving resection, chemotherapy and radiation is used for the treatment of such tumours. Immunohistochemistry lung mass primary pulmonary synovial sarcoma Figures Figure 1 Figure 2 BACKGROUND Approximately 5–10% of all soft-tissue sarcomas are synovial sarcomas (SS), which are spindle cell sarcomas with a mesenchymal origin [ 1 ]. They are malignant soft tissue tumours with an initial indolent course. Adults between the ages of 15 and 40 are usually affected with predominance in males [ 2 ]. Despite their name, these tumours do not originate from synovial structures; instead, they typically arise in close proximity to joints. Most common location is extremities (80–95%) followed by head and neck, chest wall and infrequently in lungs (2%). Radiographs might show nothing abnormal unless a big mass or dystrophic calcification is present [ 3 ]. In this report, we present an unusual case of primary pleuro-pulmonary synovial sarcoma in a young male. CASE PRESENTATION A 25 year old male presented with breathlessness for one month, which was non progressive and right sided chest pain for one month which was diffuse, dull aching and non-radiating without palpitations or sweating. There was no history of fever, cough, wheezing or hemoptysis. On examination, he was conscious and oriented and his vital parameters were normal. On respiratory system examination, there was decreased intensity of breath sounds over right hemi thorax. Chest radiograph showed right upper and middle zone homogenous opacity [ Figure 1 a ]. His routine blood investigations were normal. His CECT (contrast enhanced computed tomography) thorax confirmed a lobulated, heterogenous lesion of size 9.2 x 7.1 cm in upper and middle zone suggestive of a solid pleural based mass in the right hemithorax, a well-defined lesion in the medial basal segment of the right lower lobe and another well-defined lobulated mass in the sub carinal region [ Fig. 1 b-d ] . Ultrasound guided core needle biopsy of the lesion was done. The histopathological examination revealed fascicles of spindle cells with uniform elongated, hyperchromatic nuclei. Herring bone pattern and hemangiopericytomatous pattern were also present [ Fig. 2 a ] . Epithelial components were not observed, and there was an absence of pleomorphism, calcification, or myxoid changes in the section studied. The features were likely suggestive of monophasic synovial sarcoma, hemangiopericytoma or fibrosarcoma. For further diagnosis regarding type of soft tissue sarcoma, immunohistochemistry staining with EMA, TLE1, CD56, CD34, S100, Ki67, BCL2 were done. It revealed BCL2, EMA and TLE 1 positivity [ Fig. 2 b-d ] hence confirming diagnosis of monophasic synovial sarcoma [Table 1] . Table-1: IHC interpretation of soft tissue sarcoma TYPE OF TUMOR EMA BCL2 TLE 1 S100 CD34 KI 67 Synovial Sarcoma + ve + ve Specific for synovial sarcoma -ve -ve Variable Hemangiopericytoma -ve Variable -ve -ve + ve Variable Fibrosarcoma -ve -ve -ve -ve -ve + ve (High) * + ve: Positive; -ve: Negative. PET (positron emission tomography) CT scan showed FDG (fluorodeoxyglucose) avid lobulated pleural based soft tissue mass in the right hemithorax with metastatic right lower lobe and pleural based nodule. There were no features of distant metastasis especially in extremities which confirmed diagnosis of primary pleuro-pulmonary synovial sarcoma. Endobrobchial ultrasound (EBUS) guided biopsy was planned to asses endobronchial spread and to assess subcarinal lesion but the EBUS facility was not available at our institution. We thought of referring the patient to specialized EBUS centre but due to financial constraints, patient and his relatives refused to undergo further investigations. Though there was no distant metastasis on PET CT scan, patient already had lesion in right lower lobe and in the sub carinal region in addition to the pleural based mass. Hence, after discussion with surgical oncologist, we came to the conclusion that tumor was inoperable and proceeded with chemotherapy. Patient underwent combination chemotherapy of Ifosfamide and Doxorubicin as advised by oncologist. Unfortunately, patient lost to follow up after third cycle of chemotherapy. DISCUSSION Synovial sarcoma represents tumours more frequently in peri-articular tissue and are rarely detected in the lungs. Pulmonary sarcomas account for just 0.5% of all primary pulmonary cancers; the two most prevalent types are synovial sarcoma and malignant fibrous histiocytoma [ 4 ]. Other rare primary lung malignancies are malignant lymphoma, carcinosarcoma, epitheloid hemangioendothelioma, hemangiopericytoma,etc [ 5 ]. Examples of primary soft tissue sarcomas of the lung are leiomyosarcoma, synovial sarcoma, spindle cell sarcoma, Kaposi sarcoma and liposarcoma. Synovial sarcoma is a spindle cell tumour of mesenchymal origin with variable epithelial cell differentiation representing 8% of all soft tissue sarcomas [ 6 ]. Primary pulmonary synovial sarcoma is an extremely aggressive tumour with a 5-year survival rate of 50%, making the prognosis for patients with this tumour extremely poor [ 7 ]. The location of the tumour mostly determines the clinical characteristics like chest discomfort, coughing, dyspnea, and rarely hemoptysis. The tumour is usually big and occasionally can occupy all of the lung tissue. Patients with primary synovial sarcoma usually do not have lymphadenopathy, calcification, pleural effusion, or cavitation. Diagnosis by endobronchial ultrasonography (EBUS) and transbronchial needle aspiration (TBNA) has become more common because of the tumour’s frequent proximity to the major airways. Biphasic and monophasic are the two primary subtypes of synovial sarcoma identified by histological analysis. As exhibited in this case, monophasic synovial sarcoma is the most prevalent type of primary synovial sarcoma. This is made up of homogeneous, rather tiny spindle cells with ovoid, pale coloring nuclei and barely noticeable nucleoli .The appearance of nuclear overlapping is caused by the sparse cytoplasm and the hazy cell boundaries with the collagenous stroma in between. Biphasic synovial sarcoma is composed of both spindle and epithelial cells. The component of the epithelium cell is characterized by ovoid nuclei and glandular-patterned cytoplasm. Synovial sarcoma is characterized by a specific chromosomal translocation producing SS18‑SSX fusion gene in more than 90% of cases. Identification of this fusion gene remains the gold standard for the diagnosis in the presence of consistent histology and immunophenotype. Cytogenetic studies of synovial sarcomas have revealed the chromosomal translocation t (x; 18) (p11; q11). This translocation fused the SYT gene from chromosome 18 to either of two homologous genes at Xp11, SSX1 or SSX2 [ 8 ]. Unfortunately we could not perform chromosomal studies on this patient due to financial constraints. Fluorescent in situ hybridization (FISH) and RT‑PCR (Reverse transcription polymerase chain reaction) techniques are commonly utilized on formalin‑fixed, paraffin‑embedded tissue blocks for identification of the translocation in routine practice. In the absence of typical histomorphology, detection of fusion oncogenes is extremely helpful in differentiating SS from other tumors. Multimodality treatment including wide excision, chemotherapy, and radiotherapy is the mainstay of therapy. Wherever feasible, wide surgical resection with tumor‑free margins remains the preferred modality of treatment followed by chemotherapy and/or radiotherapy [ 9 ]. Surgery is not recommended for tumors that are locally invasive or have metastatic disease. SS is generally considered as relatively chemosensitive tumor, and treatment with adriamycin alone or in combination with ifosfamide remains the standard chemotherapy in metastatic disease [ 9 ]. In our patient there was no distant metastasis on PET CT scan but had lesion in right lower lobe and in the sub carinal region in addition to the pleural based mass. Hence, after discussion with surgical oncologist, we came to the conclusion that tumor was inoperable and proceeded with chemotherapy but patient did not follow up after third cycle of chemotherapy. There is a serious need for an ideal therapeutic agent in SS that is more effective and less toxic. Several novel potential therapeutic targets under research are SS18‑SSX fusion oncogene, epidermal growth factor receptor, and vascular endothelial growth factor receptors are the future hope in treatment of synovial sarcoma [ 10 ]. CONCLUSION In young adults, primary pulmonary synovial sarcoma is an exceptionally rare and severe tumor. As opposed to primary tumour, lung metastases occur more frequently. Clinical imaging and histopathological examination are required to rule out other primary causes; however it is immunohistochemical staining that provides a conclusive diagnosis particularly in the case of monophasic type, since it can be easily confused with other sarcoma types. Abbreviations CT: computed tomography. IHC: Immunohistochemistry. CECT: Contrast-Enhanced Computed Tomography. EMA: Epithelial membrane antigen. CD: Cluster of Differentiation. TLE1: Transducin-like enhancer 1 (Protein). S100: Soluble (Saturated) 100 (Protein). Ki67: Antigen Kiel 67 BCL2: B-cell leukemia/lymphoma 2 (Protein). PET: positron emission tomography. FDG: fluorodeoxyglucose. EBUS: endobronchial ultrasonography. TBNA: transbronchial needle aspiration. FISH: Fluorescent in situ hybridization RT‑PCR : Reverse transcription polymerase chain reaction Declarations Acknowledgements: Not applicable . Authors’ contributions SSD wrote the initial draft of the manuscript. SSD and MSB managed the diagnosis and treatment. SSD & MSB approved the final version of the manuscript and agreed to be accountable for all aspects of the work. All authors read and approved the final manuscript. Funding: The study received no external funding . Availability of data and materials: The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request. Institutional ethical approval: Our institution does not require ethics approval for reporting individual case reports. Consent for publication: Written informed consent was obtained from the patientfor the publication of the case report and the accompanyingimages. Competing/ Conflicts of interest: There are no conflicts of interest. References Panigrahi MK, Pradhan G, Sahoo N, Mishra P, Patra S and Mohapatra PR. Primary pulmonary synovial sarcoma: A reappraisal. J Cancer Res Ther. 2018; 14(3):481-489 Steven P. Meyers. MRI of Bone and Soft Tissue Tumors and Tumor like Lesions.(2008) ISBN: 9783131354211 - Google Books Murphey M, Gibson M, Jennings B, Crespo-Rodríguez A, Fanburg-Smith J, Gajewski D. From the Archives of the AFIP: Imaging of Synovial Sarcoma with Radiologic-Pathologic Correlation. Radiographics. 2006;26(5):1543-65. Shah UB, Joshi S, Ghorpade SV, Gaikwad SN, Sundrani RM. Primary pleuro-pulmonary synovial sarcoma. Indian J Chest Dis Allied Sci. 2010;52:169–72. Sekine I. , Kodama T. , Yokose T. , Nishiwaki Y. , Suzuki K. , Goto K. et al. Rare pulmonary tumors –a review of 32 cases. Oncology 1998;55(5):431-434. Banerjee R, Bandopadhyay D, Abilash VG. Epidemiology, pathology, types and diagnosis of soft tissue sarcoma: A research review. Asian J Pharm Clin Res. 2013;6:18–25. Bunch K, Deering SH. Primary pulmonary synovial sarcoma in pregnancy. Case Rep Obstet Gynecol. 2012;2012:326031. Kawai A, Woodruff J, Healey JH, Brennan MF, Antonescu CR, Ladanyi M. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med 1998;338:153-60. Jain A, Sajeevan KV, Babu KG, Lakshmaiah KC. Chemotherapy in adult soft tissue sarcoma. Indian J Cancer 2009;46:274‑87. Wakamatsu T, Naka N, Sasagawa S, Tanaka T, Takenaka S, Araki N, et al. Deflection of vascular endothelial growth factor action by SS18‑SSX and composite vascular endothelial growth factor‑ and chemokine (C‑X‑C motif) receptor 4‑targeted therapy in synovial sarcoma. Cancer Sci 2014;105:1124‑34. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Under Review Version 1 posted Submission checks completed at journal 21 Nov, 2024 First submitted to journal 21 Nov, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {\"props\":{\"pageProps\":{\"initialData\":{\"identity\":\"rs-5206970\",\"acceptedTermsAndConditions\":true,\"allowDirectSubmit\":false,\"archivedVersions\":[],\"articleType\":\"Case Report\",\"associatedPublications\":[],\"authors\":[{\"id\":381569536,\"identity\":\"613be769-8fce-44b6-b734-8f02090a28d0\",\"order_by\":0,\"name\":\"SACHINKUMAR DOLE\",\"email\":\"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA7klEQVRIiWNgGAWjYHACNoYEBmYGPgbm4x8+gLjsxGphY2BLY5wB4jITo4UBrIXHjJmHAczGD+TbDz978HCHtRwbe1vaY5tf2+T5mBkYP3zMwa3F4EyauUHimXRjNp7Dx41z+24btjEzMEvO3IZHC0MOm0Ri2+HENom0BOncntuMQC1szLx4tMj3v4FqkX9jIG3Zc9ueoBaGG3BbeMykGX7cTiSoxeDGMzOgFpBf0pINextuJ7cxMzbj9Yt8f/IzyZ9t1nL87IcPPvjx57bt/Pbmgx8+4nMYCmBsA5MNxKoHgT+kKB4Fo2AUjIKRAgAWlUv+htPauQAAAABJRU5ErkJggg==\",\"orcid\":\"\",\"institution\":\"Dr. D. Y. Patil Medical College, Hospital and Research centre,Pimpri, Pune, Maharashtra, India – 411018\",\"correspondingAuthor\":true,\"prefix\":\"\",\"firstName\":\"SACHINKUMAR\",\"middleName\":\"\",\"lastName\":\"DOLE\",\"suffix\":\"\"},{\"id\":381569537,\"identity\":\"3b683fbe-1b5c-4bb6-b0c7-4942d4556425\",\"order_by\":1,\"name\":\"M S BARTHWAL\",\"email\":\"\",\"orcid\":\"\",\"institution\":\"Dr. D. Y. Patil Medical College, Hospital and Research centre,Pimpri, Pune, Maharashtra, India – 411018\",\"correspondingAuthor\":false,\"prefix\":\"\",\"firstName\":\"M\",\"middleName\":\"S\",\"lastName\":\"BARTHWAL\",\"suffix\":\"\"}],\"badges\":[],\"createdAt\":\"2024-10-05 04:53:12\",\"currentVersionCode\":1,\"declarations\":\"\",\"doi\":\"10.21203/rs.3.rs-5206970/v1\",\"doiUrl\":\"https://doi.org/10.21203/rs.3.rs-5206970/v1\",\"draftVersion\":[],\"editorialEvents\":[],\"editorialNote\":\"\",\"failedWorkflow\":false,\"files\":[{\"id\":70034192,\"identity\":\"ec24ce67-bc17-45fe-82a7-08b5478aa01c\",\"added_by\":\"auto\",\"created_at\":\"2024-11-27 17:03:02\",\"extension\":\"png\",\"order_by\":1,\"title\":\"Figure 1\",\"display\":\"\",\"copyAsset\":false,\"role\":\"figure\",\"size\":163072,\"visible\":true,\"origin\":\"\",\"legend\":\"\\u003cp\\u003e\\u003cstrong\\u003e(a) \\u003c/strong\\u003eChest radiograph suggestive of right upper and middle zone homogenous opacity. CECT thorax revealed \\u003cstrong\\u003e(b)\\u003c/strong\\u003e well-defined lesion in the medial basal segment of the right lower lobe, \\u003cstrong\\u003e(c)\\u003c/strong\\u003e lobulated, heterogenous pleural based mass in the right hemi thorax and \\u003cstrong\\u003e(d) \\u003c/strong\\u003eanother well-defined lobulated mass in the sub carinal region.\\u003c/p\\u003e\",\"description\":\"\",\"filename\":\"floatimage2.png\",\"url\":\"https://assets-eu.researchsquare.com/files/rs-5206970/v1/076f079608c8751e89748bf3.png\"},{\"id\":70034193,\"identity\":\"971296c6-31c9-41f5-bdf8-f17124501fc2\",\"added_by\":\"auto\",\"created_at\":\"2024-11-27 17:03:02\",\"extension\":\"png\",\"order_by\":2,\"title\":\"Figure 2\",\"display\":\"\",\"copyAsset\":false,\"role\":\"figure\",\"size\":230626,\"visible\":true,\"origin\":\"\",\"legend\":\"\\u003cp\\u003e\\u003cstrong\\u003e(a) \\u003c/strong\\u003eHistopathological examination revealed fascicles of spindle cells with uniform elongated, hyperchromatic nuclei with absence of pleomorphism, calcification or myxoid changes (Hematoxylin and eosin, 40x.). Immunohistochemical staining showing positivity for (\\u003cstrong\\u003eb) \\u003c/strong\\u003eTLE1(200X). \\u003cstrong\\u003e(c) \\u003c/strong\\u003eBCL2 (200X). (\\u003cstrong\\u003ed) \\u003c/strong\\u003eEMA (200X).\\u003c/p\\u003e\",\"description\":\"\",\"filename\":\"floatimage3.png\",\"url\":\"https://assets-eu.researchsquare.com/files/rs-5206970/v1/b3fc199e65aa589b259da7a1.png\"},{\"id\":70034196,\"identity\":\"90c87306-9e3e-4795-b2bf-fa21d95595f5\",\"added_by\":\"auto\",\"created_at\":\"2024-11-27 17:03:07\",\"extension\":\"pdf\",\"order_by\":0,\"title\":\"\",\"display\":\"\",\"copyAsset\":false,\"role\":\"manuscript-pdf\",\"size\":711504,\"visible\":true,\"origin\":\"\",\"legend\":\"\",\"description\":\"\",\"filename\":\"manuscript.pdf\",\"url\":\"https://assets-eu.researchsquare.com/files/rs-5206970/v1/f070a643-a5af-405f-9f07-0d8705fe5ab5.pdf\"}],\"financialInterests\":\"No competing interests reported.\",\"formattedTitle\":\"\\u003cp\\u003eAn Unusual Case of Primary Pulmonary Synovial Sarcoma\\u003c/p\\u003e\",\"fulltext\":[{\"header\":\"BACKGROUND\",\"content\":\"\\u003cp\\u003eApproximately 5\\u0026ndash;10% of all soft-tissue sarcomas are synovial sarcomas (SS), which are spindle cell sarcomas with a mesenchymal origin [\\u003cspan citationid=\\\"CR1\\\" class=\\\"CitationRef\\\"\\u003e1\\u003c/span\\u003e]. They are malignant soft tissue tumours with an initial indolent course. Adults between the ages of 15 and 40 are usually affected with predominance in males [\\u003cspan citationid=\\\"CR2\\\" class=\\\"CitationRef\\\"\\u003e2\\u003c/span\\u003e]. Despite their name, these tumours do not originate from synovial structures; instead, they typically arise in close proximity to joints. Most common location is extremities (80\\u0026ndash;95%) followed by head and neck, chest wall and infrequently in lungs (2%). Radiographs might show nothing abnormal unless a big mass or dystrophic calcification is present [\\u003cspan citationid=\\\"CR3\\\" class=\\\"CitationRef\\\"\\u003e3\\u003c/span\\u003e]. In this report, we present an unusual case of primary pleuro-pulmonary synovial sarcoma in a young male.\\u003c/p\\u003e\"},{\"header\":\"CASE PRESENTATION\",\"content\":\"\\u003cp\\u003eA 25 year old male presented with breathlessness for one month, which was non progressive and\\u003c/p\\u003e \\u003cp\\u003eright sided chest pain for one month which was diffuse, dull aching and non-radiating without\\u003c/p\\u003e \\u003cp\\u003epalpitations or sweating. There was no history of fever, cough, wheezing or hemoptysis. On examination, he was conscious and oriented and his vital parameters were normal. On respiratory system examination, there was decreased intensity of breath sounds over right hemi thorax. Chest radiograph showed right upper and middle zone homogenous opacity \\u003cb\\u003e[\\u003c/b\\u003eFigure \\u003cspan refid=\\\"Fig1\\\" class=\\\"InternalRef\\\"\\u003e1\\u003c/span\\u003ea\\u003cb\\u003e].\\u003c/b\\u003e His routine blood investigations were normal. His CECT (contrast enhanced computed tomography) thorax confirmed a lobulated, heterogenous lesion of size 9.2 x 7.1 cm in upper and middle zone suggestive of a solid pleural based mass in the right hemithorax, a well-defined lesion in the medial basal segment of the right lower lobe and another well-defined lobulated mass in the sub carinal region \\u003cb\\u003e[\\u003c/b\\u003eFig.\\u0026nbsp;\\u003cspan refid=\\\"Fig1\\\" class=\\\"InternalRef\\\"\\u003e1\\u003c/span\\u003eb-d\\u003cb\\u003e]\\u003c/b\\u003e.\\u003c/p\\u003e \\u003cp\\u003e \\u003c/p\\u003e \\u003cp\\u003eUltrasound guided core needle biopsy of the lesion was done. The histopathological examination revealed fascicles of spindle cells with uniform elongated, hyperchromatic nuclei. Herring bone pattern and hemangiopericytomatous pattern were also present \\u003cb\\u003e[\\u003c/b\\u003eFig.\\u0026nbsp;\\u003cspan refid=\\\"Fig2\\\" class=\\\"InternalRef\\\"\\u003e2\\u003c/span\\u003ea\\u003cb\\u003e]\\u003c/b\\u003e. Epithelial components were not observed, and there was an absence of pleomorphism, calcification, or myxoid changes in the section studied. The features were likely suggestive of monophasic synovial sarcoma, hemangiopericytoma or fibrosarcoma. For further diagnosis regarding type of soft tissue sarcoma, immunohistochemistry staining with EMA, TLE1, CD56, CD34, S100, Ki67, BCL2 were done. It revealed BCL2, EMA and TLE 1 positivity \\u003cb\\u003e[\\u003c/b\\u003eFig.\\u0026nbsp;\\u003cspan refid=\\\"Fig2\\\" class=\\\"InternalRef\\\"\\u003e2\\u003c/span\\u003eb-d\\u003cb\\u003e]\\u003c/b\\u003e hence confirming diagnosis of monophasic synovial sarcoma \\u003cb\\u003e[Table\\u0026nbsp;1]\\u003c/b\\u003e.\\u003c/p\\u003e \\u003cp\\u003e \\u003c/p\\u003e \\u003cp\\u003e \\u003cb\\u003eTable-1: IHC interpretation of soft tissue sarcoma\\u003c/b\\u003e \\u003c/p\\u003e \\u003cp\\u003e \\u003cdiv class=\\\"gridtable\\\"\\u003e\\u003ctable float=\\\"No\\\" id=\\\"Tabb\\\" border=\\\"1\\\"\\u003e \\u003ccolgroup cols=\\\"7\\\"\\u003e \\u003cdiv align=\\\"left\\\" class=\\\"colspec\\\" colname=\\\"c1\\\" colnum=\\\"1\\\"\\u003e\\u003c/div\\u003e \\u003cdiv align=\\\"left\\\" class=\\\"colspec\\\" colname=\\\"c2\\\" colnum=\\\"2\\\"\\u003e\\u003c/div\\u003e \\u003cdiv align=\\\"left\\\" class=\\\"colspec\\\" colname=\\\"c3\\\" colnum=\\\"3\\\"\\u003e\\u003c/div\\u003e \\u003cdiv align=\\\"left\\\" class=\\\"colspec\\\" colname=\\\"c4\\\" colnum=\\\"4\\\"\\u003e\\u003c/div\\u003e \\u003cdiv align=\\\"left\\\" class=\\\"colspec\\\" colname=\\\"c5\\\" colnum=\\\"5\\\"\\u003e\\u003c/div\\u003e \\u003cdiv align=\\\"left\\\" class=\\\"colspec\\\" colname=\\\"c6\\\" colnum=\\\"6\\\"\\u003e\\u003c/div\\u003e \\u003cdiv align=\\\"left\\\" class=\\\"colspec\\\" colname=\\\"c7\\\" colnum=\\\"7\\\"\\u003e\\u003c/div\\u003e \\u003cthead\\u003e \\u003ctr\\u003e \\u003cth align=\\\"left\\\" colname=\\\"c1\\\"\\u003e \\u003cp\\u003eTYPE OF TUMOR\\u003c/p\\u003e \\u003c/th\\u003e \\u003cth align=\\\"left\\\" colname=\\\"c2\\\"\\u003e \\u003cp\\u003eEMA\\u003c/p\\u003e \\u003c/th\\u003e \\u003cth align=\\\"left\\\" colname=\\\"c3\\\"\\u003e \\u003cp\\u003eBCL2\\u003c/p\\u003e \\u003c/th\\u003e \\u003cth align=\\\"left\\\" colname=\\\"c4\\\"\\u003e \\u003cp\\u003eTLE 1\\u003c/p\\u003e \\u003c/th\\u003e \\u003cth align=\\\"left\\\" colname=\\\"c5\\\"\\u003e \\u003cp\\u003eS100\\u003c/p\\u003e \\u003c/th\\u003e \\u003cth align=\\\"left\\\" colname=\\\"c6\\\"\\u003e \\u003cp\\u003eCD34\\u003c/p\\u003e \\u003c/th\\u003e \\u003cth align=\\\"left\\\" colname=\\\"c7\\\"\\u003e \\u003cp\\u003eKI 67\\u003c/p\\u003e \\u003c/th\\u003e \\u003c/tr\\u003e \\u003c/thead\\u003e \\u003ctbody\\u003e \\u003ctr\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c1\\\"\\u003e \\u003cp\\u003e\\u003cb\\u003eSynovial Sarcoma\\u003c/b\\u003e\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c2\\\"\\u003e \\u003cp\\u003e\\u003cb\\u003e+ ve\\u003c/b\\u003e\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c3\\\"\\u003e \\u003cp\\u003e\\u003cb\\u003e+ ve\\u003c/b\\u003e\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c4\\\"\\u003e \\u003cp\\u003e\\u003cb\\u003eSpecific for\\u003c/b\\u003e\\u003c/p\\u003e \\u003cp\\u003e\\u003cb\\u003esynovial sarcoma\\u003c/b\\u003e\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c5\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c6\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c7\\\"\\u003e \\u003cp\\u003eVariable\\u003c/p\\u003e \\u003c/td\\u003e \\u003c/tr\\u003e \\u003ctr\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c1\\\"\\u003e \\u003cp\\u003e\\u003cb\\u003eHemangiopericytoma\\u003c/b\\u003e\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c2\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c3\\\"\\u003e \\u003cp\\u003eVariable\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c4\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c5\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c6\\\"\\u003e \\u003cp\\u003e\\u003cb\\u003e+ ve\\u003c/b\\u003e\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c7\\\"\\u003e \\u003cp\\u003eVariable\\u003c/p\\u003e \\u003c/td\\u003e \\u003c/tr\\u003e \\u003ctr\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c1\\\"\\u003e \\u003cp\\u003e\\u003cb\\u003eFibrosarcoma\\u003c/b\\u003e\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c2\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c3\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c4\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c5\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c6\\\"\\u003e \\u003cp\\u003e-ve\\u003c/p\\u003e \\u003c/td\\u003e \\u003ctd align=\\\"left\\\" colname=\\\"c7\\\"\\u003e \\u003cp\\u003e\\u003cb\\u003e+ ve (High)\\u003c/b\\u003e\\u003c/p\\u003e \\u003c/td\\u003e \\u003c/tr\\u003e \\u003c/tbody\\u003e \\u003c/colgroup\\u003e \\u003c/table\\u003e\\u003c/div\\u003e \\u003c/p\\u003e \\u003cp\\u003e* + ve: Positive; -ve: Negative.\\u003c/p\\u003e \\u003cp\\u003ePET (positron emission tomography) CT scan showed FDG (fluorodeoxyglucose) avid lobulated pleural based soft tissue mass in the right hemithorax with metastatic right lower lobe and pleural based nodule. There were no features of distant metastasis especially in extremities which confirmed diagnosis of primary pleuro-pulmonary synovial sarcoma. Endobrobchial ultrasound (EBUS) guided biopsy was planned to asses endobronchial spread and to assess subcarinal lesion but the EBUS facility was not available at our institution. We thought of referring the patient to specialized EBUS centre but due to financial constraints, patient and his relatives refused to undergo further investigations. Though there was no distant metastasis on PET CT scan, patient already had lesion in right lower lobe and in the sub carinal region in addition to the pleural based mass. Hence, after discussion with surgical oncologist, we came to the conclusion that tumor was inoperable and proceeded with chemotherapy. Patient underwent combination chemotherapy of Ifosfamide and Doxorubicin as advised by oncologist. Unfortunately, patient lost to follow up after third cycle of chemotherapy.\\u003c/p\\u003e\"},{\"header\":\"DISCUSSION\",\"content\":\"\\u003cp\\u003eSynovial sarcoma represents tumours more frequently in peri-articular tissue and are rarely\\u003c/p\\u003e \\u003cp\\u003edetected in the lungs. Pulmonary sarcomas account for just 0.5% of all primary pulmonary\\u003c/p\\u003e \\u003cp\\u003ecancers; the two most prevalent types are synovial sarcoma and malignant fibrous\\u003c/p\\u003e \\u003cp\\u003ehistiocytoma [\\u003cspan citationid=\\\"CR4\\\" class=\\\"CitationRef\\\"\\u003e4\\u003c/span\\u003e]. Other rare primary lung malignancies are malignant lymphoma, carcinosarcoma, epitheloid hemangioendothelioma, hemangiopericytoma,etc [\\u003cspan citationid=\\\"CR5\\\" class=\\\"CitationRef\\\"\\u003e5\\u003c/span\\u003e]. Examples of primary soft tissue sarcomas of the lung are leiomyosarcoma, synovial sarcoma, spindle cell sarcoma, Kaposi sarcoma and liposarcoma. Synovial sarcoma is a spindle cell tumour of mesenchymal origin with variable epithelial cell differentiation representing 8% of all soft tissue sarcomas [\\u003cspan citationid=\\\"CR6\\\" class=\\\"CitationRef\\\"\\u003e6\\u003c/span\\u003e]. Primary pulmonary synovial sarcoma is an extremely aggressive tumour with a 5-year survival rate of 50%, making the prognosis for patients with this tumour extremely poor [\\u003cspan citationid=\\\"CR7\\\" class=\\\"CitationRef\\\"\\u003e7\\u003c/span\\u003e]. The location of the tumour mostly determines the clinical characteristics like chest discomfort, coughing, dyspnea, and rarely hemoptysis. The tumour is usually big and occasionally can occupy all of the lung tissue. Patients with primary synovial sarcoma usually do not have lymphadenopathy, calcification, pleural effusion, or cavitation. Diagnosis by endobronchial ultrasonography (EBUS) and transbronchial needle aspiration (TBNA) has become more common because of the tumour\\u0026rsquo;s frequent proximity to the major airways. Biphasic and monophasic are the two primary subtypes of synovial sarcoma identified by histological analysis. As exhibited in this case, monophasic synovial sarcoma is the most prevalent type of primary synovial sarcoma. This is made up of homogeneous, rather tiny spindle cells with ovoid, pale coloring nuclei and barely noticeable nucleoli .The appearance of nuclear overlapping is caused by the sparse cytoplasm and the hazy cell boundaries with the collagenous stroma in between. Biphasic synovial sarcoma is composed of both spindle and epithelial cells. The component of the epithelium cell is characterized by ovoid nuclei and glandular-patterned cytoplasm.\\u003c/p\\u003e \\u003cp\\u003eSynovial sarcoma is characterized by a specific chromosomal translocation producing SS18‑SSX fusion gene in more than 90% of cases. Identification of this fusion gene remains the gold standard for the diagnosis in the presence of consistent histology and immunophenotype. Cytogenetic studies of synovial sarcomas have revealed the chromosomal translocation t (x; 18) (p11; q11). This translocation fused the SYT gene from chromosome 18 to either of two homologous genes at Xp11, SSX1 or SSX2 [\\u003cspan citationid=\\\"CR8\\\" class=\\\"CitationRef\\\"\\u003e8\\u003c/span\\u003e]. Unfortunately we could not perform chromosomal studies on this patient due to financial constraints.\\u003c/p\\u003e \\u003cp\\u003eFluorescent in situ hybridization (FISH) and RT‑PCR (Reverse transcription polymerase chain reaction) techniques are commonly utilized on formalin‑fixed, paraffin‑embedded tissue blocks for identification of the translocation in routine practice. In the absence of typical histomorphology, detection of fusion oncogenes is extremely helpful in differentiating SS from other tumors.\\u003cdiv class=\\\"BlockQuote\\\"\\u003e\\u003cp\\u003eMultimodality treatment including wide excision, chemotherapy, and radiotherapy is the mainstay of therapy. Wherever feasible, wide surgical resection with tumor‑free margins remains the preferred modality of treatment followed by chemotherapy and/or radiotherapy [\\u003cspan citationid=\\\"CR9\\\" class=\\\"CitationRef\\\"\\u003e9\\u003c/span\\u003e]. Surgery is not recommended for tumors that are locally invasive or have metastatic disease. SS is\\u003c/p\\u003e\\u003cp\\u003egenerally considered as relatively chemosensitive tumor, and treatment with adriamycin alone or in combination with ifosfamide remains the standard chemotherapy in metastatic disease [\\u003cspan citationid=\\\"CR9\\\" class=\\\"CitationRef\\\"\\u003e9\\u003c/span\\u003e].\\u003c/p\\u003e\\u003c/div\\u003e\\u003c/p\\u003e \\u003cp\\u003eIn our patient there was no distant metastasis on PET CT scan but had lesion in right lower lobe and in the sub carinal region in addition to the pleural based mass. Hence, after discussion with surgical oncologist, we came to the conclusion that tumor was inoperable and proceeded with chemotherapy but patient did not follow up after third cycle of chemotherapy.\\u003cdiv class=\\\"BlockQuote\\\"\\u003e\\u003cp\\u003eThere is a serious need for an ideal therapeutic agent in SS that is more effective and less toxic. Several novel potential therapeutic targets under research are SS18‑SSX fusion oncogene, epidermal growth factor receptor, and vascular endothelial growth factor receptors are the future hope in treatment of synovial sarcoma [\\u003cspan citationid=\\\"CR10\\\" class=\\\"CitationRef\\\"\\u003e10\\u003c/span\\u003e].\\u003c/p\\u003e\\u003c/div\\u003e\\u003c/p\\u003e\"},{\"header\":\"CONCLUSION\",\"content\":\"\\u003cp\\u003eIn young adults, primary pulmonary synovial sarcoma is an exceptionally rare and severe tumor. As opposed to primary tumour, lung metastases occur more frequently. Clinical imaging and histopathological examination are required to rule out other primary causes; however it is immunohistochemical staining that provides a conclusive diagnosis particularly in the case of monophasic type, since it can be easily confused with other sarcoma types.\\u003c/p\\u003e\"},{\"header\":\"Abbreviations\",\"content\":\"\\u003cul\\u003e\\n \\u003cli\\u003eCT: computed tomography.\\u003c/li\\u003e\\n \\u003cli\\u003eIHC: Immunohistochemistry.\\u003c/li\\u003e\\n \\u003cli\\u003eCECT: Contrast-Enhanced Computed Tomography.\\u003c/li\\u003e\\n \\u003cli\\u003eEMA: Epithelial membrane antigen.\\u003c/li\\u003e\\n \\u003cli\\u003eCD: Cluster of Differentiation.\\u003c/li\\u003e\\n \\u003cli\\u003eTLE1: Transducin-like enhancer 1 (Protein).\\u003c/li\\u003e\\n \\u003cli\\u003eS100: Soluble (Saturated) 100 (Protein).\\u003c/li\\u003e\\n \\u003cli\\u003eKi67: Antigen Kiel 67\\u003c/li\\u003e\\n \\u003cli\\u003eBCL2: B-cell leukemia/lymphoma 2 (Protein).\\u003c/li\\u003e\\n \\u003cli\\u003ePET: positron emission tomography.\\u003c/li\\u003e\\n \\u003cli\\u003eFDG: fluorodeoxyglucose.\\u003c/li\\u003e\\n \\u003cli\\u003eEBUS: endobronchial ultrasonography.\\u003c/li\\u003e\\n \\u003cli\\u003eTBNA: transbronchial needle aspiration.\\u003c/li\\u003e\\n \\u003cli\\u003eFISH: Fluorescent in situ hybridization\\u0026nbsp;\\u003c/li\\u003e\\n \\u003cli\\u003eRT‑PCR : Reverse transcription polymerase chain reaction\\u003c/li\\u003e\\n\\u003c/ul\\u003e\"},{\"header\":\"Declarations\",\"content\":\"\\u003cp\\u003e\\u003cstrong\\u003eAcknowledgements:\\u0026nbsp;\\u003c/strong\\u003eNot applicable\\u003cstrong\\u003e.\\u003c/strong\\u003e\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eAuthors\\u0026rsquo; contributions\\u003c/strong\\u003e\\u003c/p\\u003e\\n\\u003cp\\u003eSSD wrote the initial draft of the manuscript. SSD and MSB managed the diagnosis and treatment. SSD \\u0026amp; MSB approved the final version of the manuscript and agreed to be accountable for all aspects of the work. All authors read and approved the final manuscript.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eFunding:\\u0026nbsp;\\u003c/strong\\u003eThe study received no external funding\\u003cstrong\\u003e.\\u003c/strong\\u003e\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eAvailability of data and materials:\\u0026nbsp;\\u003c/strong\\u003eThe datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eInstitutional ethical approval:\\u0026nbsp;\\u003c/strong\\u003eOur institution does not require ethics approval for reporting individual case reports.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eConsent for publication:\\u0026nbsp;\\u003c/strong\\u003eWritten informed consent was obtained from the patientfor the publication of the case report and the accompanyingimages.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eCompeting/ Conflicts of interest:\\u0026nbsp;\\u003c/strong\\u003eThere are no conflicts of interest.\\u003c/p\\u003e\\n\"},{\"header\":\"References\",\"content\":\"\\u003col\\u003e\\n\\u003cli\\u003ePanigrahi MK, Pradhan G, Sahoo N, Mishra P, Patra S and Mohapatra PR. Primary pulmonary synovial sarcoma: A reappraisal. J Cancer Res Ther. 2018; 14(3):481-489\\u003c/li\\u003e\\n\\u003cli\\u003eSteven P. Meyers. MRI of Bone and Soft Tissue Tumors and Tumor like Lesions.(2008) ISBN: 9783131354211 - Google Books\\u003c/li\\u003e\\n\\u003cli\\u003eMurphey M, Gibson M, Jennings B, Crespo-Rodr\\u0026iacute;guez A, Fanburg-Smith J, Gajewski D. From the Archives of the AFIP: Imaging of Synovial Sarcoma with Radiologic-Pathologic Correlation. Radiographics. 2006;26(5):1543-65. \\u003c/li\\u003e\\n\\u003cli\\u003eShah UB, Joshi S, Ghorpade SV, Gaikwad SN, Sundrani RM. Primary pleuro-pulmonary synovial sarcoma. Indian J Chest Dis Allied Sci. 2010;52:169\\u0026ndash;72.\\u003c/li\\u003e\\n\\u003cli\\u003eSekine I. , Kodama T. , Yokose T. , Nishiwaki Y. , Suzuki K. , Goto K. et al. Rare pulmonary tumors \\u0026ndash;a review of 32 cases. Oncology 1998;55(5):431-434.\\u003c/li\\u003e\\n\\u003cli\\u003eBanerjee R, Bandopadhyay D, Abilash VG. Epidemiology, pathology, types and diagnosis of soft tissue sarcoma: A research review. Asian J Pharm Clin Res. 2013;6:18\\u0026ndash;25.\\u003c/li\\u003e\\n\\u003cli\\u003eBunch K, Deering SH. Primary pulmonary synovial sarcoma in pregnancy. Case Rep Obstet Gynecol. 2012;2012:326031.\\u003c/li\\u003e\\n\\u003cli\\u003eKawai A, Woodruff J, Healey JH, Brennan MF, Antonescu CR, Ladanyi M. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med 1998;338:153-60.\\u003c/li\\u003e\\n\\u003cli\\u003eJain A, Sajeevan KV, Babu KG, Lakshmaiah KC. Chemotherapy in adult soft tissue sarcoma. Indian J Cancer 2009;46:274‑87.\\u003c/li\\u003e\\n\\u003cli\\u003eWakamatsu T, Naka N, Sasagawa S, Tanaka T, Takenaka S, Araki N, et al. Deflection of vascular endothelial growth factor action by SS18‑SSX and composite vascular endothelial growth factor‑ and chemokine (C‑X‑C motif) receptor 4‑targeted therapy in synovial sarcoma. Cancer Sci 2014;105:1124‑34.\\u003c/li\\u003e\\n\\u003c/ol\\u003e\"}],\"fulltextSource\":\"\",\"fullText\":\"\",\"funders\":[],\"hasAdminPriorityOnWorkflow\":false,\"hasManuscriptDocX\":true,\"hasOptedInToPreprint\":true,\"hasPassedJournalQc\":\"\",\"hasAnyPriority\":false,\"hideJournal\":false,\"highlight\":\"\",\"institution\":\"\",\"isAcceptedByJournal\":true,\"isAuthorSuppliedPdf\":false,\"isDeskRejected\":\"\",\"isHiddenFromSearch\":false,\"isInQc\":false,\"isInWorkflow\":false,\"isPdf\":false,\"isPdfUpToDate\":true,\"isWithdrawnOrRetracted\":false,\"journal\":{\"display\":true,\"email\":\"info@researchsquare.com\",\"identity\":\"the-egyptian-journal-of-bronchology\",\"isNatureJournal\":false,\"hasQc\":true,\"allowDirectSubmit\":false,\"externalIdentity\":\"\",\"sideBox\":\"Learn more about [The Egyptian Journal of Bronchology](https://ejb.springeropen.com/)\",\"snPcode\":\"43168\",\"submissionUrl\":\"https://submission.nature.com/new-submission/43168/3\",\"title\":\"The Egyptian Journal of Bronchology\",\"twitterHandle\":\"\",\"acdcEnabled\":true,\"dfaEnabled\":true,\"editorialSystem\":\"stoa\",\"reportingPortfolio\":\"Springer Open\",\"inReviewEnabled\":true,\"inReviewRevisionsEnabled\":true},\"keywords\":\"Immunohistochemistry, lung mass, primary pulmonary synovial sarcoma\",\"lastPublishedDoi\":\"10.21203/rs.3.rs-5206970/v1\",\"lastPublishedDoiUrl\":\"https://doi.org/10.21203/rs.3.rs-5206970/v1\",\"license\":{\"name\":\"CC BY 4.0\",\"url\":\"https://creativecommons.org/licenses/by/4.0/\"},\"manuscriptAbstract\":\"\\u003cp\\u003e\\u003cstrong\\u003eBackground\\u003c/strong\\u003e: An extremely rare intrathoracic tumour that typically affects young adults is primary pulmonary synovial\\u003cstrong\\u003e \\u003c/strong\\u003esarcoma. It manifests as a big intrathoracic mass without involvement of the bone or\\u003cstrong\\u003e \\u003c/strong\\u003emediastinal region. Tumour biopsy, immunostaining and histologic analysis can assist in determining the kind of tumour.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eCase Presentation\\u003c/strong\\u003e: We present a case of a 25 year old male with complaints of breathlessness and chest pain for a month. On radiological imaging he was found to have mass in upper and middle zones of right hemithorax. For confirmation of diagnosis patient underwent ultrasound guided biopsy of the lesion and histopathologhical examination (HPE) of the lesion. The histopathological features were suggestive of a rare type of lung malignancy. For further diagnosis regarding type of malignancy, immunohistochemical staining of the lesion was done with the help of with specific immunological markers, which confirmed the diagnosis of monophasic pulmonary sarcoma. On PET (positron emission tomography) CT (computed tomography) scan there were no features suggestive of distant metastasis especially in extremities which confirmed the diagnosis of primary pulmonary synovial sarcoma. Patient was started on chemotherapy regimen of Ifosfamide and Doxorubicin after oncologist’s opinion but was lost to follow up after 3\\u003csup\\u003erd\\u003c/sup\\u003e cycle of chemotherapy.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eConclusions\\u003c/strong\\u003e: Primary pulmonary synovial sarcoma is an extremely uncommon tumor in young adults as lung metastases from other sources mainly extremities are more common. Diagnosis of such rare tumour requires histopathological examination along with immunohistochemical staining of the lung lesions. A multimodal strategy of treatment involving resection, chemotherapy and radiation is used for the treatment of such tumours.\\u003c/p\\u003e\",\"manuscriptTitle\":\"An Unusual Case of Primary Pulmonary Synovial Sarcoma\",\"msid\":\"\",\"msnumber\":\"\",\"nonDraftVersions\":[{\"code\":1,\"date\":\"2024-11-27 17:02:57\",\"doi\":\"10.21203/rs.3.rs-5206970/v1\",\"editorialEvents\":[{\"type\":\"communityComments\",\"content\":0},{\"type\":\"checksComplete\",\"content\":\"\",\"date\":\"2024-11-21T13:23:59+00:00\",\"index\":\"\",\"fulltext\":\"\"},{\"type\":\"submitted\",\"content\":\"The Egyptian Journal of Bronchology\",\"date\":\"2024-11-21T06:04:59+00:00\",\"index\":\"\",\"fulltext\":\"\"}],\"status\":\"published\",\"journal\":{\"display\":true,\"email\":\"info@researchsquare.com\",\"identity\":\"the-egyptian-journal-of-bronchology\",\"isNatureJournal\":false,\"hasQc\":true,\"allowDirectSubmit\":false,\"externalIdentity\":\"\",\"sideBox\":\"Learn more about [The Egyptian Journal of Bronchology](https://ejb.springeropen.com/)\",\"snPcode\":\"43168\",\"submissionUrl\":\"https://submission.nature.com/new-submission/43168/3\",\"title\":\"The Egyptian Journal of Bronchology\",\"twitterHandle\":\"\",\"acdcEnabled\":true,\"dfaEnabled\":true,\"editorialSystem\":\"stoa\",\"reportingPortfolio\":\"Springer Open\",\"inReviewEnabled\":true,\"inReviewRevisionsEnabled\":true}}],\"origin\":\"\",\"ownerIdentity\":\"8872886f-21f4-467e-bb10-f14801b61e26\",\"owner\":[],\"postedDate\":\"November 27th, 2024\",\"published\":true,\"recentEditorialEvents\":[],\"rejectedJournal\":[],\"revision\":\"\",\"amendment\":\"\",\"status\":\"under-review\",\"subjectAreas\":[],\"tags\":[],\"updatedAt\":\"2024-11-27T17:02:57+00:00\",\"versionOfRecord\":[],\"versionCreatedAt\":\"2024-11-27 17:02:57\",\"video\":\"\",\"vorDoi\":\"\",\"vorDoiUrl\":\"\",\"workflowStages\":[]},\"version\":\"v1\",\"identity\":\"rs-5206970\",\"journalConfig\":\"researchsquare\"},\"__N_SSP\":true},\"page\":\"/article/[identity]/[[...version]]\",\"query\":{\"redirect\":\"/article/rs-5206970\",\"identity\":\"rs-5206970\",\"version\":[\"v1\"]},\"buildId\":\"qtupq5eGEP_6zYnWcrvyt\",\"isFallback\":false,\"isExperimentalCompile\":false,\"dynamicIds\":[84888],\"gssp\":true,\"scriptLoader\":[]}","source_license":"CC-BY-4.0","license_restricted":false}