SESSION TITLE: Disorders of the Pleura Global Case Reports
SESSION TYPE: Global Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Synovial sarcoma is a soft-tissue tumor that most commonly affects the extremities near large joints in young and middle-aged adults. However, synovial sarcomas are also reported to occur in other parts of the body, such as the head and neck, mediastinum, heart, lungs, pleura, mesentery, and retroperitoneal space. Primary synovial sarcoma of the pleura is rare, and its prognosis and treatment are not well defined. The benefits of chemotherapy for primary synovial sarcoma of the pleura are unclear but improvement in survival has been reported with doxorubicin-ifosfamide combination chemotherapy. We report a case of rapidly progressed primary pleural synovial sarcoma, treated with doxorubicin and ifosfamide in combination as first-line chemotherapy, and pazopanib (a tyrosine kinase inhibitor) as second-line chemotherapy.
CASE PRESENTATION: A 42-year-old woman presented at the outpatient clinic of the Osaka City University Hospital with a history of chest pain and breathlessness. CT scan of the chest demonstrated a nodule measuring 2×2 cm at the right lung base. The patient presented at the emergency department after 2 weeks, complaining of difficulty breathing. A CT scan demonstrated enlargement of the tumor, and the occurrence of a right pleural effusion. Video-assisted thoracic surgery (VATS) demonstrated giant mass at right lower lobe, hemorrhagic pleural effusion and pleural dissemination. Biopsy specimen showed the presence of both epithelial component with high cellularity and short spindle cell with fibrous proliferation, proving biphasic type. Immunohistochemical analyses revealed positive staining for CD99 and vimentin, and focal staining for B-cell lymphoma 2 (Bcl-2) and cytokeratins. However, mesothelium markers (thrombomoduline, HBME1), markers of lung cancer (TTF1, Napsin A) and neuroendocrine markers (NSE,Chromogranin A) were negative. Although chimeric mRNA qualitative analysis by reverse transcription-polymerase chain reaction (RT-PCR) assays was performed on RNA extracted from the paraffin block, the product of the SYT-SSX fusion gene was not detected. According to immunohistochemical analyses, we diagnosed it as pleural synovial sarcoma (biphasic type). As systemic metastasis to the pelvis was detected on positron emission tomography (PET) before starting therapy, the patient was treated with a combination of doxorubicin (60 mg/m2) and ifosfamide (10 g/m2) as first-line chemotherapy. Doxorubicin-ifosfamide combination chemotherapy achieved a transient decrease in tumor size; however, an increase in tumor size was observed later. The patient was treated with the tyrosine kinase inhibitor, pazopanib as second-line chemotherapy. However, pazopanib had little therapeutic effect, and the pleural synovial sarcoma progressed up to the patient’s death.
DISCUSSION: Pleural synovial sarcoma is a rare tumor, which Gaertner et al. reported the first case in 1996[Reference #1]. Most pleural synovial sarcoma patients are young or middle-aged adults, and there is no sex bias. Although the SYT-SSX fusion gene is found in approximately 90% of synovial sarcomas of the extremities, frequency of the SYT-SSX fusion gene in pleural synovial sarcoma is unknown. Even if molecular testing for SYT-SSX fusion gene was negative, the diagnosis of synovial sarcoma is made by immunohistochemistry. Only after 14 days, tumor got greater and massive pleural effusion occurred. Systemic chemotherapy was selected in our case because of metastasis to the pelvis and pleural dissemination. Although the combination of doxorubicin and ifosfamide initially resulted in a significant reduction in the tumor size, the tumor size increased again. Our case then received second-line chemotherapy with pazopanib, a potent and selective multi-targeted receptor tyrosine kinase inhibitor. It was well tolerated, with the minor adverse effect of hair discoloration. Although pazopanib showed a transient anti-tumor effect with stable tumor size, tumor progression was observed later.
CONCLUSIONS: We have reported a case of rapidly progressed and pazopanib-treated pleural synovial sarcoma. For the improvement of pleural synovial sarcoma treatment, further data regarding its natural history, diagnosis, and treatment are needed.
Reference #1: Gaertner E, Zeren EH, Fleming MV, Colby TV, Travis WD. Biphasic synovial sarcomas arising in the pleural cavity: A clinicopathologic study of five cases. Am J Surg Pathol 20:36-45, 1996.
DISCLOSURE: Kazuhisa Asai: Consultant fee, speaker bureau, advisory committee, etc.: lecture fee Kazuto Hirata: Consultant fee, speaker bureau, advisory committee, etc.: Lecture fee The following authors have nothing to disclose: Kanako Sato, Yuko Kubo, Atsuko Okamoto, Kazuhiro Yamada, Naoko Yoshii, Tetsuya Watanabe, Kazuhisa Konishi, Masato Uji
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