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Introduction. Pleural synovial sarcoma (SS) is a rare type of mesenchymal tumor, that can easily be misdiagnosed. Case report. We presented a case of primary monophasic SS of the pleura in a middle-aged woman who initially presented with dyspnoea and a large pleural effusion. Computed tomography (CT) scans showed a large, well-demarcated right lung tumor mass. After a blind closed biopsy of the pleura, the tumor was misdiagnosed as adenocarcinoma and treated with chemotherapy but without response. The correct diagnosis was established after surgery and histological and immunohistochemical analyses. The diagnosis was fulfilled with cytogenetic analysis showing the typical translocation t (X,18). The tumor was completely extirpated during surgery. CT of the chest done four, and positron emission tomography done six months after surgery showed encapsulated re-active pleural effusion without tumor rest or relapse. In contrast, a CT scan done nine months after surgery showed an extrapulmonary soft-tissue mass in contact with the lower right lobe highly suspicious of tumor relapse. Surgery was performed, and the described mass was extirpated, but histological analysis showed no presence of malignant tissue. CT scan performed three months later showed no signs of the disease relapse. Conclusion. Considering that pleural SS can easily be misdiagnosed, immunohistochemical as well as cytogenetic analysis should always be performed in order to reach the proper diagnosis.

Primary pleural synovial sarcoma (SS) is an extremely rare entity, especially in the group of children and adolescents. Due to its rarity in this location and variable histogenesis, the diagnosis of SS is a clinical challenge. We report a new case of primary pleural SS in an adolescent. A 14-year-old boy initially referred to another facility for spontaneous right-sided chest pain and dyspnea, and was transferred to our hospital five days later for recurrent hemorrhagic pleural effusion in the right chest cavity. The items in routine haematological investigations were within normal limits, except for the lower level of eosinophil. CT scan revealed a heterogeneously enhancing solid mass occupied the upper two-thirds of the right hemithorax and massive pleural effusion in the lower half of the hemithorax. There was no evidence of hilar or mediastinal lymphadenopathy. Subsequently, an open thoracotomy was performed and the tumour was diagnosed as biphasic SS based on morphological and immunohistochemical analysis. The patient was discharged after surgical resection without complications and adjuvant chemotherapy was arranged. Currently, the patient is clinically well 6 months after surgery, with no evidence of recurrent disease. Though pleural SS is a rare anatomic subset of SS, it should be considered in the differential diagnosis of intrathoracic mass without hilar or mediastinal lymphadenopathy in adolescents, especially the cases with recurrent hemorrhagic pleural effusion.

Synovial sarcoma (SS) primary to the pleura is an extremely rare tumor. So far, only nine cases have been reported in pediatric patients. However, none of these patients was found to have a conglomeration of tumors. Here, we report a case of 16-year-old female with monophasic SS and synchronous occurrence of left paraspinal ganglioneuroma and a right paraovarian cystadenoma. A next-generation sequencing genetic panel revealed a novel variant of unknown significance in the MET gene. The occurrence of multiple different tumors in a young patient with a novel genetic variant in a known oncogene (MET) may suggest a possibility of a hitherto unknown cancer predisposition syndrome. We also present a brief review of primary pleural SS reported in pediatric patients.

The mediastinum is a rare site for occurrence of a primary synovial sarcoma (SS) with very few cases reported in the literature. The diagnosis so far has been achieved mainly via open surgery, with only three reported cases diagnosed via endobronchial ultrasound with transbronchial needle aspiration (EBUS-TBNA), however, none of those were located at the posterior mediastinum, without showing any oesophageal or endobronchial involvement. To our knowledge, this is the first reported case of a monophasic primary pleural mediastinal SS purely involving the posterior mediastinum without oesophageal or endobronchial component, diagnosed via EBUS-TBNA.

Pleural effusion is a common condition in respiratory medicine. According to the criteria established by Light...

Primary pleural synovial sarcoma (PPSS) is a rare malignant pleural tumor comprising < 1% of all primary lung malignancies. Primary pleural mesothelioma (PPM) has many similar features that may cause a diagnostic dilemma due to overlapping clinical and histopathological features. We present the case of a young male with recurrent hemorrhagic pleural effusion without any obvious lung mass who was diagnosed with PPSS. This rare entity must be considered with a high index of suspicion while evaluating pleural tumors.

Primary pleural synovial sarcoma (PPSS) is an extremely rare malignancy without a known cause. The diagnosis is made after excluding metastasis from an extra‐thoracic sarcoma. We report a case of a 67‐year‐old gentleman who presented with an incidental finding of a left lung mass on a routine chest X‐ray. A computed tomography (CT) of the thorax and whole‐body positron emission tomography (PET)‐CT was done confirming a left lung mass with no other extra‐thoracic involvement. A lobectomy was performed with a diagnostic and therapeutic intent. The histopathological examination and immunohistochemistry study revealed a pleural‐based tumour with features suggestive of synovial sarcoma. Subsequently, he underwent post‐operative radiotherapy. However, three months later, he developed an endobronchial recurrence, complicated by post‐obstructive pneumonia resulting in his demise. This case highlights a rare form of malignancy with a rare site of recurrence., Primary pleural synovial sarcoma (PPSS) is an extremely rare malignancy without a known cause. We report a case of a PPSS that was operated and recurred as an endobronchial metastasis.

Primary tumors of the pleura are relatively rare both in children and adults. Metastatic disease to the pleura is the most common neoplastic process involving the pleural surfaces and far outnumbers primary benign and malignant pleural neoplasms. Malignant mesothelioma represents the most common primary malignant neoplasm of this site, with an estimated 2000 newly diagnosed cases in the United States each year [Cagle and Allen, Respirology 16:430–438, 2011; English and Leslie, Clin Chest Med 27:157–180, 2006; Ai and Stevenson, Oncologist 19:975–984, 2014]. Aside from malignant mesothelioma, there is a variety of more uncommon primary pleural neoplasms, both benign and malignant, that need to be considered in the differential diagnosis. Since many of these entities can demonstrate similar clinical, imaging, and macroscopic features, awareness of these conditions as well as accurate histopathologic diagnosis is extremely important as the treatment and prognosis for these diverse tumors can vary greatly. Novel immunohistochemical and molecular markers allow for a confirmatory diagnosis in some of the entities; however, careful morphologic assessment and evaluation of the clinical context are indispensable for the correct diagnosis, not only to distinguish between different primary pleural neoplasms but also to exclude metastatic disease. This chapter provides an overview of the most common primary pleural neoplasms other than malignant mesothelioma and reviews their typical clinical presentation as well as morphologic, immunophenotypic, and molecular characteristics.

Rationale: Apatinib is an oral tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor-2. It has been shown that apatinib is effective and safe for treatment of multiple solid tumors, including gastric cancer, liver cancer, non-small-cell lung cancer, and breast cancer. However, there is currently no consensus as to using Apatinib for the treatment of pleural synovial sarcoma, due to the rarity of primary pleural synovial sarcoma and lack of clinical studies as a consequence. Patient concerns and diagnoses: We reported here in the case of a 26-year-old Chinese woman diagnosed with pleural synovial sarcoma. She has undergone 2 surgeries, multiple regimens of chemotherapy and traditional Chinese medicine in other hospitals. Then the patient was admitted to our hospital with the compliant of chest pain and dyspnea. The medical history and available data supported the diagnosis of recurrence of pleural synovial sarcoma. Interventions and outcomes: Due to the lack of efficacy of previous standard treatment, the patient was given apatinib and radiotherapy to relieve the symptoms. This patient achieved stable disease with apatinib at a dose of 500 mg/day. Her progression-free survival time was more than 7 months, and her overall survival was 8.5 months. Except for hand-foot syndrome, no grade 3 or 4 side effects were observed. Conclusions: Apatinib may thus be an option for treatment of advanced synovial sarcoma after failure of other treatments. However, further study is needed to determine the efficacy of apatinib in pleural synovial sarcoma.

Rationale Apatinib is an oral tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor-2. It has been shown that apatinib is effective and safe for treatment of multiple solid tumors, including gastric cancer, liver cancer, non-small-cell lung cancer, and breast cancer. However, there is currently no consensus as to using Apatinib for the treatment of pleural synovial sarcoma, due to the rarity of primary pleural synovial sarcoma and lack of clinical studies as a consequence. Patient concerns and diagnoses We reported here in the case of a 26-year-old Chinese woman diagnosed with pleural synovial sarcoma. She has undergone 2 surgeries, multiple regimens of chemotherapy and traditional Chinese medicine in other hospitals. Then the patient was admitted to our hospital with the compliant of chest pain and dyspnea. The medical history and available data supported the diagnosis of recurrence of pleural synovial sarcoma. Interventions and outcomes Due to the lack of efficacy of previous standard treatment, the patient was given apatinib and radiotherapy to relieve the symptoms. This patient achieved stable disease with apatinib at a dose of 500 mg/day. Her progression-free survival time was more than 7 months, and her overall survival was 8.5 months. Except for hand-foot syndrome, no grade 3 or 4 side effects were observed. Conclusions Apatinib may thus be an option for treatment of advanced synovial sarcoma after failure of other treatments. However, further study is needed to determine the efficacy of apatinib in pleural synovial sarcoma.

Primary pleural synovial sarcoma is a malignant tumour and thought to be more aggressive than synovial sarcoma which occurs in extremities. Its treatment strategy has not been fully established because of its rarity. We report a primary pleural synovial sarcoma case which achieved a long-term survival with repeated surgery of recurrent pleural tumour. A 39-year-old man presented with a gradually enlarged tumour in the left hemithorax. The tumour was resected and diagnosed as primary pleural synovial sarcoma. The tumour was slowly growing and repeatedly recurrent in the left pleura. The surgical resections for the recurrent tumours were performed 6 years and 11 years after the initial surgery. Intriguingly, recurrent tumour which developed after second surgery exhibited temporally spontaneous regression. Our patient remains alive 12 years after the initial surgery. Repeated resection of metastatic lesion can achieve long survival in primary pleural synovial sarcoma.

Primary pleural sarcomas constitute

Pleural malignant mesothelioma (MM) includes several unusual and even rare but distinctive histological subtypes, in addition to the usual subdivision into epithelioid, biphasic and sarcomatoid MM. Criteria for discrimination between fibrous pleuritis versus desmoplastic mesothelioma include evidence of neoplastic invasion for diagnosis of desmoplastic MM, but this histological assessment is complicated by the recently-described 'fake fat phenomenon' in cases of fibrous pleuritis. The distinction between biphasic and monophasic synovial sarcoma of the pleura versus biphasic and sarcomatoid MM can be problematical and is most cogently based upon molecular detection of the t(X;18) translocation, whereas a clear diagnosis of MM for a pleural tumour histologically resembling synovial sarcoma is favoured by a negative result for this translocation and, probably, microRNA evidence supportive of a diagnosis of MM. Aquaporin-1 (AQP1) is a molecule involved in the growth of MM cells, and yet is a factor reported to correlate with improved survival rates for MM with an epithelioid component, in comparison to AQP1-poor MM, as assessed from AQP1 expression by epithelioid MM cells only (apart from co-expression by stromal endothelial cells in addition to the tumour cells). Recent reports have also focused upon germline mutations in the BRCA1-associated protein 1 (BAP1), not only in cases of familial mesothelioma, but also BAP1 deletion in sporadic MM. Prognostic factors for MM include not only the histological subtypes, but other independent variables that include (among others), AQP1 expression by mesothelioma cells, the clinical status of the patient, the serum neutrophil:lymphocyte ratio and blood thrombocytosis.

Primary pleuropulmonary synovial sarcomas are rare soft tissue malignancies; combined metastatic involvement of the heart is extremely rare. In this case report, a 17-year-old female presented with a history of chest pain. Chest radiographs revealed a round mass in the left upper hemithorax, and computed tomography (CT) showed a well-defined heterogeneous enhancing mass abutting the pleura. A core needle biopsy revealed malignant spindle cells. Surgical resection was performed, and a final diagnosis of primary pleural synovial sarcoma, monophasic fibrous type, was made. The patient underwent radical irradiation and chemotherapy and remained stable for 28 months until a follow-up chest CT showed a poorly enhancing nodule in the left pericardial region that enlarged after 5 months. Surgical resection was performed. Histological examination confirmed metastatic cardiac involvement from a primary pleural synovial sarcoma. We report this unusual case of a primary pleural synovial sarcoma metastasis to the heart.

The article presents the rare case of a 64-year-old woman, who was admitted to our thoracic surgery department with a giant tumor in a right hemithorax measuring 88 mm × 137 mm × 188 mm, revealed by a thoracic CT scan. An anterolateral thoracotomy with a radical tumor resection was performed. The final pathological diagnosis of the poorly differentiated synovial sarcoma (PDSS) was made. The adjuvant radiotherapy of 60 Gy in 30 fractions was applied postoperatively. One year after operation patient remains in good health. The literature review on pleural synovial sarcoma has been shortly presented.

金沢大学医薬保健研究域医学系, 背景．胸膜および肺に原発する滑膜肉腫は非常に稀である．症例．35歳，男性．右肺の異常陰影の精査目的に当院に紹介された．胸郭内原発の悪性腫瘍が疑われ，当院呼吸器外科にて胸腔鏡を行った．右胸腔内は脆弱な腫瘍で埋め尽くされていた．病理学的に，腫瘍は密に増殖する紡錘形細胞から成り，多数の核分裂像を認めた．免疫組織化学的検索を行ったが，確定診断には至らなかった．腫瘍は急速に増大し，約2ヶ月後に呼吸不全にて死亡した．剖検時，右肺は腫瘍により圧排され，虚脱していた．外科切除標本からRNAを抽出し，reverse transcription polymerase chain reaction（RT-PCR）を行い，さらにダイレクトシークエンスを行い，PCR産物がSYT-SSX2 融合遺伝子転写産物であることを確認した．胸膜以外に腫瘍を疑わせる所見を認めなかった．以上より，胸膜原発の滑膜肉腫と診断した．結論．非常に稀な腫瘍である胸膜原発の滑膜肉腫の1例を報告した．Background. Primary synovial sarcoma of the pleura and lung is extremely rare. Case. A 35-year-old man was admitted to our hospital because of an abnormal opacity in the right lung. A malignant intrathoracic tumor was suspected and a video-assisted thoracoscopic biopsy was performed. The right thoracic cavity was occupied by a fragile tumor. Histologically, the tumor showed dense proliferation of spindle cells with high rate of mitosis. Immunohistochemical examination was performed, but no definitive diagnosis was obtained. Progression of the tumor was very rapid and he died of respiratory failure 2 months after first presentation. On autopsy, the right lung was compressed and collapsed by the tumor. An SYT-SSX2 fusion gene transcript was detected by reverse transcription polymerase chain reaction (RT-PCR) and direct sequencing using RNA extracted from resected tissue specimens. There was no evidence of tumor except in the pleura. The final diagnosis was primary pleural synovial sarcoma. Conclusion. We report an extremely rare case of primary pleural synovial sarcoma. © 2010 The Japan Lung Cancer Society.

We describe a rare case of primary pleural synovial sarcoma in a 27-year-old man with a 4-month history of dry cough and left-sided chest pain. A CT scan showed a large cystic mass in the left pleural cavity. The patient underwent two video-assisted thoracoscopic biopsies and the diagnosis of synovial sarcoma of the pleura was established. After neoadjuvant chemotherapy, which resulted in a partial response, the tumor was completely resected with extrapleural pneumonectomy. Pathological findings showed less than 5% of viable cancer and free surgical margins. The patient is clinically well 24 months after surgery, with no evidence of recurrent disease.

背景．胸膜および肺に原発する滑膜肉腫は非常に稀である．症例．35歳，男性．右肺の異常陰影の精査目的に当院に紹介された．胸郭内原発の悪性腫瘍が疑われ，当院呼吸器外科にて胸腔鏡を行った．右胸腔内は脆弱な腫瘍で埋め尽くされていた．病理学的に，腫瘍は密に増殖する紡錘形細胞から成り，多数の核分裂像を認めた．免疫組織化学的検索を行ったが，確定診断には至らなかった．腫瘍は急速に増大し，約2ヶ月後に呼吸不全にて死亡した．剖検時，右肺は腫瘍により圧排され，虚脱していた．外科切除標本からRNAを抽出し，reverse transcription polymerase chain reaction（RT-PCR）を行い，さらにダイレクトシークエンスを行い，PCR産物がSYT-SSX2 融合遺伝子転写産物であることを確認した．胸膜以外に腫瘍を疑わせる所見を認めなかった．以上より，胸膜原発の滑膜肉腫と診断した．結論．非常に稀な腫瘍である胸膜原発の滑膜肉腫の1例を報告した．

Primary pleural synovial sarcoma is a rare disease with poor outcomes. Although hyperthermia therapy as part of a combined treatment regimen can offer improved local tumor control, only two reports of hyperthermia therapy for synovial sarcoma have appeared in the literature, and these sarcomas were not of pleuropulmonary origin. This report of an advanced inoperable primary pleural synovial sarcoma is the first to address the use of hyperthermia therapy in combination with chemoradiotherapy for this disease, together with radiological assessment following that therapy. Computed tomography performed after thermoradiation showed a decrease in tumor size and a characteristic unenhanced low-density area in the tumor suggesting that tumor necrosis resulted from the therapy. These image findings were helpful in assessing the tumor response to thermoradiation. We believe that hyperthermia therapy combined with chemoradiotherapy should be regarded as an option for advanced primary pleural synovial sarcoma. This would give computed tomography important role in evaluating this approach.

A 42-year-old woman presented with chest pain and breathlessness with a nodule measuring 2×2 cm in size at the base of the right lung. A bronchoscopic examination did not reveal any malignancy. However, the patient developed difficulty in breathing, enlargement of the nodule, and right pleural effusion 14 days later. A video-assisted thoracic surgical biopsy specimen revealed the presence of pleural synovial sarcoma. The patient was treated with doxorubicin-ifosfamide combination chemotherapy because of metastasis to the pelvis. However, after a transient partial clinical response, there was a relapse of refractory disease. Although treated with pazopanib as second-line chemotherapy, the patient died eight months after the initial presentation.

The origin of a ring chromosome in a monophasic synovial sarcoma of the diaphragmatic pleura of an 18-year-old man was investigated using spectral karyotyping (SKY) and fluorescence in situ hybridization (FISH). Conventional cytogenetic analysis revealed the following clonal karyotypic abnormalities: 47,Y,t(X;18)(p11.2;q11.2),t(11;19)(q12;q13.4),+12,-13,+r[6]. The SYT-SSX1 fusion transcript was detected with reverse transcriptase-polymerase chain reaction analysis. SKY analysis suggested that the ring chromosome was composed of material from chromosome 8. Subsequent FISH analysis with a whole-chromosome 8 paint probe confirmed the SKY results. This study demonstrates the usefulness of SKY as an adjunct for determining the chromosomal composition of ring chromosomes.

Synovial sarcoma is a malignant soft tissue tumor that most commonly occurs in the extremities of young and middle-aged adults, in the vicinity of large joints. Although synovial sarcoma is frequently associated with joints, it may arise in unexpected sites, such as the mediastinum, heart, lung, pleura, or chest wall. Primary synovial sarcoma of the pleura is rare. To date, nearly 36 cases of primary synovial sarcoma of the pleura have been reported since Gaertner et al. published the first case in 1996. The oncologic characteristics, treatment, and prognosis for pleural synovial sarcomas are not well defined because of a paucity of data. However, a multimodal approach, including surgical resection, chemotherapy, and radiotherapy, has generally been suggested. We report the outcome of one patient with primary pleural synovial sarcoma treated with radical resection and adjuvant treatment.

In this article, progress in distinguishing mesothelioma from mesothelial hyperplasia and fibrosing pleuritis was discussed. Advances in the immunohistochemical characterization of mesothelioma, in the recognition of entities that mimic mesothelioma, and in their distinction from mesothelioma were reviewed. Cytogenetic and molecular genetic contributions to the diagnosis of pleural synovial sarcoma were briefly summarized. The diagnosis of epithelial type mesothelioma can be established in most cases. Several mesotheliomas, however, especially rare subtypes, and sarcomatoid, desmoplastic, and poorly differentiated mesotheliomas continue to present diagnostic challenges.

Synovial sarcoma (SS) is an uncommon soft tissue tumor that occurs primarily in the extremities of young adults, especially in the periarticular region. In this report, we describe the rare occurrence of primary SS of the pleura in a 15-year-old boy. Histologically, the tumor demonstrated monophasic morphologic findings and showed positive staining with vimentin and Bcl-2 and focally for cytokeratin CK7. Fluorescent in situ hybridization identified t(X;18) translocation. The patient developed recurrences 20 months following resection of the tumor. The literature on this uncommon entity is reviewed, and its histogenesis, differential diagnoses, and cytologic features are also discussed.

Synovial sarcoma is a rare, highly malignant soft-tissue tumor that occurs primarily in the extremities. At present, there is no effective clinical treatment for this condition. The present study reports the case of a 49-year-old male who was diagnosed with pleural synovial sarcoma and treated with recombinant human endostatin (Endostar) combined with chemotherapy for a total of six cycles, followed by sunitinib maintenance therapy. To the best of our knowledge, this is the first reported use of sunitinib for maintenance therapy in pleural synovial sarcoma. The patient survived for 25 months after the recurrence of the disease following surgery. The results indicate that this combination therapy was effective in the treatment of pleural synovial sarcoma. The present study also briefly reviews the literature on pleural synovial sarcoma, and the features and treatments for this rare case are discussed.

Aims: Synovial sarcomas are rare tumours occasionally arising in the pleural cavity, a site where their histological characteristics may be mistaken for those of malignant mesothelioma. We examined three cases of primary pleural synovial sarcoma in order to look for clinicopathological features that may help in distinguishing them from both mesotheliomas and other sarcomas that may arise in the pleura. Methods and results All three patients were male, aged 42, 28 and 42, respectively, and had no known exposure to asbestos. One biphasic tumour contained neutral mucin in focal epithelial elements that also stained positively for BerEP4 and AUA1. All three tumours showed focal positivity for either keratin or EMA in the sarcomatous elements, and they also stained positively for bcl-2 protein and MIC2 gene product (CD99). Conclusions Our results emphasize the importance of being aware of synovial sarcoma as a possible primary pleural malignancy, especially in a young patient with a localized mass. In addition, the presence of bcl-2 protein perhaps represents a useful marker in distinguishing synovial sarcoma, especially monophasic variants, from mesothelioma within a panel of antibodies.

Percutaneous radiofrequency (RF) thermal ablation has received attention as an effective minimally invasive approach for the treatment of a variety of neoplasms. However, the therapeutic efficacy of RF thermal ablation on primary pleural synovial sarcoma has not yet been reported. A 76-year-old man with a primary pleural synovial sarcoma who was medically inoperable received RF thermal ablation to achieve local control. Therefore, an RF electrode was inserted into the lesion and connected to an RF generator. In this report, a case of successful treatment of a primary pleural synovial sarcoma using RF ablation without complication is presented.

Primary pleural synovial sarcoma (PPSS) is a rare pleural malignancy with a grave prognosis. Most cases present as a well-circumscribed mass with foci of haemorrhage and necrosis. We present an unusual case in a Nepalese boy that presented as a multiloculated cyst mimicking hydatid disease. The diagnosis was confirmed by histology and cytogenetic analysis.

Synovial sarcomas are rare soft tissue malignancies that most commonly affect the extremities in the vicinity of large joints. These malignancies typically occur in adolescents and young adults between the ages of 15 and 40 years.1,2 Historically they are believed to originate from primitive pluripotent mesenchyme capable of synovial differentiation. This belief is consistent with the malignancy's origin from sites devoid of normal synovium, such as the pleural cavity. A variety of pleural cavity sarcomas have been described, including liposarcoma,3 chondrosarcoma,4 osteosarcoma,5 and malignant schwannoma.6 Pleural synovial sarcoma, however, is a much rarer entity. In fact, pleural synovial sarcoma was first described only 6 years ago7 and has not yet been reported in the surgical literature. Because of its rarity, pleural synovial sarcoma is often mistaken for the histologically similar malignant mesothelioma, the most common of the pleural neoplasms. This is a critical distinction, because synovial sarcoma may be extremely aggressive. Studies in the last 10 years have shown it to be extremely sensitive to ifosfamide-based chemotherapy, and survival of patients with synovial sarcoma has recently increased with chemotherapy, with 5-year survivals now as high as 57%.8-10 In this report, we describe 3 cases of synovial sarcoma of the pleura. Clinical findings are correlated with pathologic features, including immunohistochemical stains and fluorescence in situ hybridization (FISH) for the identification of the diagnostic chromosomal translocation, t(X;18)(p11.2;q11.2). This delineation of the clinical and pathologic aspects of this rare, newly recognized tumor should increase awareness among the surgical community.J Thorac Cardiovasc Surg 2002;124:828-32

This study reports five cases of primary pleural monophasic synovial sarcomas and assesses the role of the SYT-SSX fusion transcript in the differential diagnosis. Patients had a mean age of 47 years with no gender predilection. Chest pain and pleural-based masses with effusions characterized the clinical presentations. Each patient underwent a complete surgical resection of the mass. The mean follow-up was 9 months, available in four patients. They were all alive, with no evidence of disease. Histologically, neoplasms were composed of densely packed fusiform cells focally alternating with less cellular areas. No epithelial differentiation was identified at the hematoxylin and eosin level. Keratin and epithelial membrane antigen reactivity was focal and present in four and two tumors, respectively. There was no immunoreactivity for CD34. RT-PCR studies for the presence of a SYT-SSX1 or SYT-SSX2 fusion transcript were positive in every tumor. In comparison, 10 localized fibrous tumors were immunohistochemically negative for keratin and epithelial membrane antigen and positive for CD34. A SYT-SSX fusion transcript was not identified in any of five localized fibrous tumors tested. Identification of the synovial sarcoma-specific chimeric transcript (SYT-SSX1 or SYT-SSX2), in conjunction with immunoperoxidase studies, can be extremely helpful in identifying cases of pleural monophasic synovial sarcoma.

This is a case report of a young adult presenting with hemorrhagic pleural effusion. Chest CT scan showed loculated pleural effusion with pleural nodule. Whole body PET scan showed thickening of pleura with multiple enhancing pleural nodules with different metabolic activity. Pleural nodule was biopsied which on histopathology showed pleural synovial sarcoma.