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BRIEF COMMUNICATION
Year : 2016  |  Volume : 12  |  Issue : 2  |  Page : 1098-1101

Multimodality management of primary diaphragmatic synovial sarcoma: First report


1 Institute of Pulmonology, Medical Research and Development; Lung Care and Sleep Centre, Fortis Hospitals, Mumbai, Maharashtra, India
2 Department of Oncosurgery, Fortis Hospital Mulund, Mumbai, Maharashtra, India
3 Department of Plastic Surgery, Fortis Hospital Mulund, Mumbai, Maharashtra, India
4 Department of Radiology, Fortis Hospital Mulund, Mumbai, Maharashtra, India
5 Department of General Surgery and Laparoscopy, Fortis Hospital Mulund, Mumbai, Maharashtra, India
6 Department of Pathology, SRL Diagnostics, Mumbai, Maharashtra, India
7 Department of Oncology, Fortis Hospital Mulund, Mumbai, Maharashtra, India

Date of Web Publication25-Jul-2016

Correspondence Address:
Prashant N Chhajed
Institute of Pulmonology, Medical Research and Development, B/24, Datta Apartments, Ramkrishna Mission Marg, 15th Road, Khar West, Mumbai - 400 052, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.179525

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 > Abstract 


Synovial cell sarcoma is an extremely rare tumor of mesenchymal origin. It commonly affects the soft tissues of the extremities but could possibly origin from the head and neck, heart, lung, pleura, mediastinum, esophagus, abdominal wall and the mesentery, and retroperitoneum. Primary synovial sarcoma of pleura, mediastinum, and lung have been reported. Primary synovial sarcoma of the diaphragm has not been reported to the best of our knowledge. We report a case of primary synovial cell sarcoma of the diaphragm presenting as a recurrent pleural effusion and pain in the left hypochondrium managed with multimodality approach.

Keywords: Diaphragmatic reconstruction surgery, hemorrhagic pleural effusion, monophasic synovial sarcoma, primary synovial sarcoma of diaphragm, thoracic synovial sarcoma


How to cite this article:
Vaidya PJ, Heroor A, Prasad S, Bhargava R, Arulvannan N, Mehta J, Dhabhar BN, Chhajed PN. Multimodality management of primary diaphragmatic synovial sarcoma: First report. J Can Res Ther 2016;12:1098-101

How to cite this URL:
Vaidya PJ, Heroor A, Prasad S, Bhargava R, Arulvannan N, Mehta J, Dhabhar BN, Chhajed PN. Multimodality management of primary diaphragmatic synovial sarcoma: First report. J Can Res Ther [serial online] 2016 [cited 2019 Oct 18];12:1098-101. Available from: http://www.cancerjournal.net/text.asp?2016/12/2/1098/179525




 > Introduction Top


Synovial cell sarcoma is an extremely rare tumor of mesenchymal origin.[1] It commonly affects the soft tissues of the extremities but could possibly origin from the head and neck, heart, lung, pleura, mediastinum, esophagus, abdominal wall and the mesentery, and retroperitoneum.[1] It carries a poor prognosis.[2] Primary synovial sarcoma of pleura, mediastinum, and lung have been reported. Primary synovial sarcoma of the diaphragm has not been reported to the best of our knowledge. We report a case of primary synovial cell sarcoma of the diaphragm presenting as a recurrent pleural effusion and pain in the left hypochondrium.


 > Case Report Top


A 46-year-old male presented with recurrent left-sided exudative pleural effusion, persistent left lumbar, and hypochondriac pain and weight loss of 10 kg over 3 months. Pleural fluid cytochemistry revealed lymphocytic exudate with normal adenosine deaminase levels. Pleuroscopy revealed a lost sheen of the parietal pleura and hemorrhagic effusion in the pleural cavity with no obvious abnormality. Pleuroscopic parietal pleural biopsy histopathology revealed inflammation without any evidence of malignancy. Subsequently, a positron emission tomography with computed tomography (CT) of the whole body revealed a metabolic active retrocrural lesion with minimal bilateral pleural effusion (left > right) with increased uptake in the costal pleura. The fluorodeoxyglucose uptake was evident in the homogenously enhancing retrocrural lesion measuring 6.3 cm × 4 cm with SUVmax of 2.5. Left crux of diaphragm could not be seen separately from the mass [Figure 1] and [Figure 2]. The lesion was not involving esophagus, lung or any other structure and being inseparable from the diaphragm. A CT-guided biopsy of the retrocrural lesion was done. Histopathology revealed a spindle cell lesion arranged in fascicles without necrosis [[Figure 3]. On immunohistochemistry, the tumor cells expressed EMA (extremely focal), Mic 2, bcl 2, calponin, and CD56 [Figure 4],[Figure 5],[Figure 6],[Figure 7],[Figure 8]. It was immunonegative for calretinin, cytokeratin, WT-1, S100 protein, and CD34 [Figure 9]. Hence, a diagnosis of monophasic synovial sarcoma was achieved. A surgical resection of the left crux of the diaphragm was done followed by reconstruction of the diaphragm using latissimus dorsi muscle flap. However an R0 resection [2] was not feasible due to the extension of the tumor to the vertebral canal posteriorly. The patient was ventilated for 72 h and was uneventfully weaned off. The patient recovered well postoperatively. The patient was administered chemotherapy with ifosfamide, adriamycin and mesna followed by local radiation to the postoperative bed with 6 Mv photons to a total dose of 60 Gy/30 fractions/43 days were administered.
Figure 1: Computed tomography image of thorax showing diaphragmatic mass (a)

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Figure 2: Positron emission tomography image of thorax showing uptake of diaphragmatic mass (b)

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Figure 3: (a and b) Histopathology microscopy high power view showing spindle cells arranged in fascicles without necrosis

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Figure 4: Focal EMA positivity on immunohistochemistry microscopic view

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Figure 5: Mic2 positive uptake on immunohistochemistry microscopic view

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Figure 6: Bcl2 positive uptake on immunohistochemistry microscopic view

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Figure 7: Calponin positive uptake on immunohistochemistry microscopic view

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Figure 8: CD56 positive uptake on immunohistochemistry microscopic view

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Figure 9: CD34 negativity on immunohistochemistry microscopic view

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 > Discussion Top


Synovial sarcoma is a rare soft tissue tumor with an incidence of 5–10% of all soft tissue tumors.[2],[3] It most commonly involves the soft tissue from the extremities in young adults.[1],[3],[4] It is also reported to occur in the head and neck, mediastinum, lung, pleura, esophagus, abdominal wall, and mesentery.[3],[5],[6],[7],[8] It is an aggressive tumor and carries a poor prognosis.[2] The translocation t(X; 18) (p11.2; q11.2) is reported to be present in more than 90% of these neoplasms, regardless of the histological subtype and site.[2],[3] Synovial sarcoma in the thorax can present with chest pain, pleural effusion, hemoptysis, dyspnea, and cough.[1],[9]

Primary synovial sarcomas can be monophasic, biphasic or poorly differentiated based on the histogenesis.[1] The reported occurrence of this tumor in tissues other than joints with the presence of epithelial differentiation, which is absent in normal synovium support the postulation of these neoplasms arising from pluripotent mesenchyme with the potential for epithelial and spindle cell differentiation.[1],[3] The current report describes spindle cell differentiation on histopathology.

On immunohistochemistry, except for a subset of monophasic tumors, most synovial sarcoma shows positive staining with cytokeratin, EMA, vimentin, and CD99.[3] Recent studies of synovial sarcoma have described bcl-2 positivity.[3] Monophasic synovial sarcoma can occasionally show S100 protein expression.[3] Immunoreactivity for CD56 and other nerve sheath-associated markers, namely Type IV collagen, CD57, and p75NTR has also been reported.[3] Synovial sarcoma is immunonegative for CD34, desmin, and SMA.[3] In the current report, the tumor cells expressed EMA (extremely focal), Mic 2, bcl 2, calponin and CD56 and immunonegativity for calretinin, cytokeratin, WT-1, S100 protein, and CD34. Hence, a diagnosis of monophasic synovial sarcoma was made.[10]

As per the NCCN guidelines, surgical excision of the tumor with the goal of achieving tumor-free margins is considered the first line of therapy.[2] If tumor-free margins are not achieved (R0 resection) radiation therapy and adjuvant chemotherapy are to be considered.[2] Our patient underwent chemotherapy with ifosfamide, adriamycin and mesna followed by radiation for residual disease.

Spillane et al. assessed 150 patients and reported the overall 5-year survival rate for soft tissue sarcomas to be 57%.[11] They also demonstrated that age >20 years at diagnosis and the trend in size (≥5 cm) were associated with a significantly worse prognosis.[11] Our patient had both the poor prognostic factors, primary tumor >5 cm and age >20 years. Our patient is doing well at the end of therapy follow-up and working full time.

To the best of our knowledge, this is the first reported case of monophasic synovial sarcoma of the diaphragm as a cause of the hemorrhagic recurrent pleural effusion. The current report also highlights the multidisciplinary approach to the management diaphragmatic synovial sarcoma.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
 > References Top

1.
Sandeepa HS, Kate AH, Chaudhari P, Chavan V, Patole K, Lokeshwar N, et al. Primary pleural synovial sarcoma: A rare cause of hemorrhagic pleural effusion in a young adult. J Cancer Res Ther 2013;9:517-9.  Back to cited text no. 1
    
2.
von Mehren M, Benjamin RS, Bui MM, Casper ES, Conrad EU 3rd, DeLaney TF, et al. Soft tissue sarcoma, version 2.2012: Featured updates to the NCCN guidelines. J Natl Compr Canc Netw 2012;10:951-60.  Back to cited text no. 2
    
3.
Ng SB, Ahmed Q, Tien SL, Sivaswaren C, Lau LC. Primary pleural synovial sarcoma. A case report and review of the literature. Arch Pathol Lab Med 2003;127:85-90.  Back to cited text no. 3
    
4.
Henninger B, Freund M, Zelger B, Putzer D, Bonatti H, Müller L, et al. Primary mediastinal synovial sarcoma: A case report and review of the literature. Cases J 2009;2:6948.  Back to cited text no. 4
    
5.
Aparna M, Natarajan J, Arumugam C, Radhakrishnan R. Primary synovial sarcoma of the maxilla. J Cancer Res Ther 2014;10:739-41.  Back to cited text no. 5
    
6.
Rao L, Jaiprakash P, Palankar N, Gowda V. Intra-abdominal primary monophasic synovial sarcoma with hemangiopericytoma-like areas. J Cancer Res Ther 2013;9:102-4.  Back to cited text no. 6
    
7.
Pal M, Ghosh BN, Roy C, Manna AK. Posterior mediastinal biphasic synovial sarcoma in a 12 year-old boy: A case report and review of literature. J Cancer Res Ther 2010;6:564-6.  Back to cited text no. 7
    
8.
Kataria T, Janardhan N, Abhishek A, Sharan GK, Mitra S. Pulmonary metastasis from renal synovial sarcoma treated by stereotactic body radiotherapy: A case report and review of the literature. J Cancer Res Ther 2010;6:75-9.  Back to cited text no. 8
    
9.
Satoh H, Takayashiki N, Shiozawa T, Miyazaki K, Ohara G, Kagohashi K, et al. Recurrent pulmonary synovial sarcoma effectively treated with amrubicin: A case report. Exp Ther Med 2015;9:1947-9.  Back to cited text no. 9
    
10.
Roy P, Das A, Sarkar A, Dwari A, Datta S. A primary synovial sarcoma of lung. N Am J Med Sci 2012;4:241-3.  Back to cited text no. 10
    
11.
Spillane AJ, A'Hern R, Judson IR, Fisher C, Thomas JM. Synovial sarcoma: A clinicopathologic, staging, and prognostic assessment. J Clin Oncol 2000;18:3794-803.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]



 

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