Primary mediastinal synovial sarcoma: a case report and review of the literature

Henninger, Benjamin; Freund, Martin C.; Zelger, Bettina; Putzer, Daniel; Bonatti, Hugo; Müller, Ludwig; Fiegl, Michael; Geltner, Christian

doi:10.4076/1757-1626-2-6948

Cited by 10 publications

(16 citation statements)

References 13 publications

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“…Common symptoms include chest pain, shortness of breath, hemoptysis and cough. However, most patients present with a slow growing, painless mass, in a way similar to our case [6,8,13].…”

Section: Discussionsupporting

confidence: 62%

“…Although synovial sarcoma has been reported to metastasis to the mediastinum, its occurrence as primary neoplasm in this location is rare and has only recently been recognized. Only a few reports and series of primary synovial sarcoma of the mediastinum are described in the literature and all reported cases does not exceed forty so far [2][3][4][6][7][8][9][10][11][12][13][14][15].…”

Section: Discussionmentioning

confidence: 99%

“…Radiographic examination should include chest radiography, Computed Tomography and Magnetic Resonance Imaging (MRI) of the tumor. On chest radiographs, the lesion is typically uniform with well-circumscribed rounded or lobulated borders [4,6,13].…”

Section: Discussionmentioning

confidence: 99%

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Primary Synovial Sarcoma of the Mediastinum: A Case Report and Review of the Literature

Ouji¹,

Souissi²,

Mbarek³

et al. 2015

J Clin Case Rep

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Background: Synovial sarcomas most commonly occur in the soft tissues of the extremities of young adults. A primary occurrence in the mediastinum is very rare with only a few reported cases in the world literature. Study aims: to insist on the rarity of this pathology, its imaging features and the importance for recognizing unusual presentation of this aggressive neoplasm to aid appropriate clinical management. Cases: Here in, we report a rare case of primary monophasic spindle cell synovial sarcoma of the mediastinum in a 31-year-old man who had an incidental finding of such a tumor. The imaging studies on admission showed 12 cm anterior-middle mediastinal mass with multiple metastases in the lung, pleura, mediastinal lymph nodes and spine. An ultrasound-guided percutaneous needle biopsy of a cervical extension of the mass permitted histopathology and immuno histochemical analysis which supported the diagnosis of monophasic spindle cell synovial sarcoma. The patient was treated by chemo-therapy without response. Conclusion: A wide range of neoplasms both primary and metastatic occurs in the mediastinum which poses considerable diagnostic difficulties. A synovial sarcoma should be considered in the differential diagnosis. Imaging can guide percutaneous needle biopsy of the tumor and evaluate its stage. Immunohistochemistry can be very helpful for an accurate diagnosis. Figure 1: PA and lateral chest x ray show a mass of the anterior-middle mediastinum displacing the trachea to the right side with multiple metastatic pulmonary nodules.

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“…Common symptoms include chest pain, shortness of breath, hemoptysis and cough. However, most patients present with a slow growing, painless mass, in a way similar to our case [6,8,13].…”

Section: Discussionsupporting

confidence: 62%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Primary Synovial Sarcoma of the Mediastinum: A Case Report and Review of the Literature

Ouji¹,

Souissi²,

Mbarek³

et al. 2015

J Clin Case Rep

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“…When complete surgical resection is not possible due to factors such as proximity of tumor to critical structures, maximum possible resection followed by adjuvant radiotherapy is the favored treatment [ 5 ]. Synovial sarcoma is one of the few sarcoma subtypes that has a considerable sensitivity to chemotherapy with reported response rates of 30–55%.…”

Section: Discussionmentioning

confidence: 99%

Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature

Madabhavi

Kataria

Patel

et al. 2015

Case Reports in Oncological Medicine

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Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35-year-old male patient who presented with acute symptoms of dyspnoea, facial puffiness, voice-hoarseness, and engorged neck veins. With the clinical picture consistent with the superior vena cava (SVC) syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in situ hybridisation (FISH) demonstrated the t(X:18) translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three cycles of neoadjuvant chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further postoperative treatment with adjuvant chemoradiotherapy was provided. Patient currently is free of disease. This is to the best of our knowledge the first report in the world literature of a successfully treated case of “primary mediastinal sarcomas presenting as SVC syndrome.” Patient is under regular surveillance at our clinic and remains free of recurrence one year after treatment completion.

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“…Radiotherapy and adjuvant chemotherapy can enhance the likelihood of disease control. When complete surgical resection is not possible due to factors such as proximity of tumour to critical structures, maximum possible resection followed by adjuvant radiotherapy is the favored treatment .…”

Section: Discussionmentioning

confidence: 99%

Primary mediastinal synovial sarcoma with subsequent development of primary adenoid cystic carcinoma of lung presenting as superior vena cava syndrome

Madabhavi

Patel

Anand

et al. 2016

Clinical Respiratory J

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Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35 year old male patient who presented with acute symptoms of dyspnoea, facial puffiness, engorged neck veins and hoarseness of voice. With the clinical picture consistent with the superior vena caval (SVC) syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in-situ hybridization (FISH) demonstrated the t(X: 18) translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three-cycles of neo-adjuvant (ifosfamide 2400mg/m2 on days 1-5 and doxorubicin 37.5 mg/m2 on days 1 & 2) chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further post-operative treatment with adjuvant chemo-radiotherapy was provided. Patient was under regular surveillance at our clinic and remains free of symptoms one-year after treatment completion. But after 14 months of treatment completion patient again had symptoms of progressive dyspnea, hoareness of voice and mild facial puffiness over a period of 2 months. On further investigating he was found to have right-sided centrally located mass with cystic and necrotic changes with extension and compression of trachea, SVC, right upper lobe bronchus and its branches. Histopathological examination of the biopsy from the lesion revealed adenoid cystic carcinoma of the lung. Rest of the metastatic work up was within normal. Immunohistochemistry of the specimen revealed c-Kit positivity. In view of the morbid second surgery he was put on Imatinib 400mg once a day and celecoxib 200mg twice a day. After 4 months patient had partial response and presently continuing with the same regimen. Extensive literature search didn't reveal much information on combined primary mediastinal sarcoma and adenoid cystic carcinoma of lung.Please cite this paper as: Madabhavi I, Patel A, Anand A, Panchal H and Parikh S. Primary mediastinal synovial sarcoma with subsequent development of primary adenoid cystic carcinoma of lung presenting as superior vena cava syndrome. Clin Respir J 2018; 12: 306-311.

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Primary mediastinal synovial sarcoma: a case report and review of the literature

Cited by 10 publications

References 13 publications

Primary Synovial Sarcoma of the Mediastinum: A Case Report and Review of the Literature

Primary Synovial Sarcoma of the Mediastinum: A Case Report and Review of the Literature

Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature

Primary mediastinal synovial sarcoma with subsequent development of primary adenoid cystic carcinoma of lung presenting as superior vena cava syndrome

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