Large primary pleural synovial sarcoma with severe dyspnea: a case report

Our plain-English summary of Large primary pleural synovial sarcoma with severe dyspnea: a case report, by M. Yamaki, S. Yonehara, T. Noriyuki (Surgical Case Reports, 2017).

The study

• Authors: M. Yamaki, S. Yonehara, T. Noriyuki
• Journal: Surgical Case Reports
• Published: 2017;3(1):29
• Cited by: 9 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Background: Synovial sarcoma is a malignant neoplasm of soft tissues. It occurs mainly in the extremities and is closely related to tendons, tendon sheaths, and bursal structures. Primary synovial sarcoma of the pleura and lungs is extremely rare.

Case Presentation: We present the case of a 62-year-old man with a large synovial sarcoma of the left pleura. He presented with general fatigue and severe dyspnea. Chest computed tomography (CT) revealed a 20-cm tumor in the left thoracic cavity. We first diagnosed the tumor as a sarcomatoid mesothelioma based on CT-guided needle biopsy. We speculated that his severe dyspnea was because of ventilation-perfusion mismatch due to the left pulmonary collapse. Furthermore, we thought that there was a discrepancy between the CT findings and the pathological findings from the biopsy specimen. We performed pleuropneumonectomy through an anterior approach with median sternotomy and 5th-intercostal thoracotomy. The resected specimen contained a 22-cm pleural tumor with parenchymatous hemorrhage. We diagnosed the tumor as monophasic synovial sarcoma based on its morphologic and immunohistochemical features. We suspected there was microscopic residual tumor in the left diaphragm and therefore performed radiation therapy. After radiotherapy, he received adjuvant chemotherapy with ifosfamide and Adriamycin. One year after surgery, the patient is alive with no signs of tumor recurrence.

Conclusions: We report a case of a large synovial sarcoma of the pleura in a patient with severe dyspnea. He was treated with pleuropneumonectomy, radiotherapy, and adjuvant chemotherapy. Although the best treatment for this rare condition has not been defined, we thought that tumor resection and adjuvant therapy were appropriate to control the disease in this case.

In plain English

This was a case report from Japan describing one patient, a 62-year-old man who was an ex-smoker. The article states that he had no history of exposure to asbestos. He had general fatigue and severe shortness of breath. A CT scan showed a 20-cm tumor in the left chest cavity. A needle biopsy was first diagnosed as sarcomatoid mesothelioma, and the tumor grew rapidly over one month. Surgeons removed the pleura and left lung. The removed tumor measured 22 cm. Based on its appearance under the microscope and staining results, the authors diagnosed it as monophasic synovial sarcoma of the pleura, not mesothelioma. A genetic test did not find the fusion gene typical of synovial sarcoma, and one rarer variant was not tested. Because some tumor might have been left on the diaphragm, the patient received 50 Gy of radiation and then chemotherapy with ifosfamide and Adriamycin. Twelve months after surgery the patient was alive without signs of recurrence. The authors concluded that surgery plus additional therapy was appropriate in this case, while noting that the best treatment has not been defined.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (one patient)
• Who was studied: one 62-year-old man, an ex-smoker, treated by surgeons at Onomichi General Hospital, Japan
• Exposure: the article states the patient had no history of asbestos exposure; he was an ex-smoker
• Main results:
  • CT showed a 20-cm tumor in the left chest cavity
  • the CT-guided needle biopsy was first diagnosed as sarcomatoid mesothelioma
  • the tumor grew rapidly over one month
  • the resected specimen was a 22-cm pleural tumor with bleeding inside it
  • the tumor cells stained positive for CD99 and BCL-2, which the authors say are usually negative in malignant mesothelioma, making mesothelioma less likely
  • no SS18-SSX fusion gene transcript, characteristic of synovial sarcoma, was found
  • final diagnosis was monophasic synovial sarcoma, based on morphologic and immunohistochemical features
  • after surgery the patient received 50 Gy of radiation to the diaphragm, then chemotherapy with ifosfamide and Adriamycin
  • the patient was discharged on postoperative day 21, and performance status improved from 3 to 1
  • one year after surgery the patient was alive with no signs of recurrence
• The authors' conclusion: The authors concluded that, although the best treatment for this rare condition has not been defined, tumor resection and adjuvant therapy were appropriate to control the disease in this case.
• Limitations the authors state:
  • the authors did not test for a minor fusion gene variant that synovial sarcoma can have
  • the authors state that the indications for surgery in this case were controversial
  • the authors state that the optimal treatment for pleural synovial sarcoma has not been defined

Topics

Medical terms in this study, explained in our Lexicon: Synovial Sarcoma, Dyspnea, Neoplasm, Pleura, Sarcomatoid, Mesothelioma, Needle Biopsy, Biopsy.

Related studies

• Synovial sarcoma of the pleura and its differentiation from other primary pleural tumours: a clinicopathological and immunohistochemical review of three cases — A.G. Nicholson, P. Goldstraw, et al. (1998)
• TLE1 is expressed in the majority of primary pleuropulmonary synovial sarcomas — L.S. Lino-Silva, J.P. Flores-Gutiérrez, et al. (2011)
• Whole-exome sequencing in primary pulmonary synovial sarcoma misdiagnosed as mesothelioma: A case report and literature review — D. Wu, H. Zhang, et al. (2023)
• Fluoro deoxyglucose positron emission tomography-computerized tomography in primary staging and response assessment of a rare case of primary pleural synovial sarcoma — C.N. Harisankar, J. John, et al. (2015)
• A Pulmonary Zebra: Adult Primary Pulmonary Synovial Sarcoma — S. Sandra, N. Chidi, et al. (2022)
• Calretinin and other mesothelioma markers in synovial sarcoma: analysis of antigenic similarities and differences with malignant mesothelioma — M. Miettinen, J. Limon, et al. (2001)

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