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Year : 2013  |  Volume : 9  |  Issue : 3  |  Page : 517-519

Primary pleural synovial sarcoma: A rare cause of hemorrhagic pleural effusion in a young adult

1 Department of Pulmonology, Fortis Hiranandani Hospital, Vashi, Navi Mumbai, Maharashtra, India
2 Department of Radiology, Fortis Hiranandani Hospital, Vashi, Navi Mumbai, Maharashtra, India
3 Department of Pathology, SRL Diagnostics, Goregaon, Mumbai, Maharashtra, India
4 Department of Oncology, Fortis Hiranandani Hospital, Vashi, Navi Mumbai, Maharashtra, India

Date of Web Publication8-Oct-2013

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

DOI: 10.4103/0973-1482.119367

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 > Abstract 

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.

Keywords: Hemorrhagic pleural effusion, pleural nodule, synovial sarcoma

How to cite this article:
Sandeepa HS, Kate AH, Chaudhari P, Chavan V, Patole K, Lokeshwar N, Chhajed PN. Primary pleural synovial sarcoma: A rare cause of hemorrhagic pleural effusion in a young adult. J Can Res Ther 2013;9:517-9

How to cite this URL:
Sandeepa HS, Kate AH, Chaudhari P, Chavan V, Patole K, Lokeshwar N, Chhajed PN. Primary pleural synovial sarcoma: A rare cause of hemorrhagic pleural effusion in a young adult. J Can Res Ther [serial online] 2013 [cited 2020 May 26];9:517-9. Available from: http://www.cancerjournal.net/text.asp?2013/9/3/517/119367

 > Introduction Top

Synovial sarcomas are rare soft tissue malignancies that most commonly affect the extremities in the vicinity of the large joints; however, they are also reported to occur in the head and neck, mediastinum, heart, esophagus, lungs, abdominal wall, mesentery and retroperitoneum. [1] Synovial sarcoma of the pleura most often represents metastatic disease from a primary soft tissue tumor. The primary synovial sarcoma of pleura is rare and only few cases have been reported in the literature. We report a case of primary pleural synovial sarcoma of pleura presenting as hemorrhagic pleural effusion in a young adult.

 > Case Report Top

A 31-year-old male patient came to us with a history of low-grade fever, dry cough, left-sided chest pain and breathlessness since 20 days. He was an occasional cigarette smoker and used to consume alcohol three to four times a week. General physical examination was normal except for the presence of pallor. On auscultation, he had reduced breath sounds on left side. Chest X-ray showed evidence of massive pleural effusion on left side with contralateral mediastinal shift. Chest ultrasound was suggestive of moderate effusion with multiple loculations. The largest loculation was along the left lateral chest wall. An intercostal tube drain was introduced on left side in view of recurrently filling effusion. Routine hematological investigations were normal. Pleural fluid analysis showed exudative effusion with neutrophilia. Pleural fluid ADA was 13.8 IU/L (0.02-36.00 IU/L) and pleural fluid cytology was negative for malignant cells. Pleural fluid TB PCR was negative. CT scan of thorax was suggestive of loculated pleural effusion on left side along with evidence of enhancing pleural nodule [Figure 1]. CT-guided biopsy of pleural nodule was suggestive of spindle cell tumor on histopathology. On immunohistochemistry CD34 was negative, MIC-2 was positive, smooth muscle actin was negative, Ki-67 was positive (30%) and Bcl-2 was positive [Figure 2], [Figure 3], [Figure 4] and [Figure 5]. The final diagnosis was synovial sarcoma of monophasic type. Whole body PET scan showed thickening of pleura with multiple enhancing pleural nodules showing different metabolic activity [Figure 6]. Two subcentimeter-sized nodules were seen in the left upper and lower lobes, which did not show any metabolic activity. There was no evidence of metabolically active disease elsewhere in the body. The patient was started on ifosfamide and adriamycin combination chemotherapy. He has received three cycles of chemotherapy and is doing well at present on follow-up.
Figure 1: CT scan of thorax showing loculated pleural effusion with a pleural-based nodular lesion (arrow)

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Figure 2: Atypical spindle cells in clusters and cords with area of necrosis and hemorrhage (hematoxylin-eosin, original magnification ×400)

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Figure 3: Diffuse immunoreactivity for Bcl-2 in neoplastic cells. (Immunohistochemical stain, original magnification ×400)

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Figure 4: Membranous immunoreactivity for MIC-2 in neoplastic cells. (Immunohistochemical stain, original magnification ×400)

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Figure 5: CD34 immunostaining negative in neoplastic cells and positive in vascular wall. (Immunohistochemical stain, original magnification ×400)

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Figure 6: PET CT scan showing pleural nodules (arrow) with increased uptake

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 > Discussion Top

Primary pleural synovial sarcomas (PPSS) are rare neoplasms that have been described in literature over the past 15 years. The first case was published by Gaertner et al. in 1996. [2] The tumor is believed to arise from pleuripotent mesenchymal cells rather than synovial cells per se because they occur in places such as pleura where synovial cells do not exist. Primary synovial sarcomas can be classified as monophasic, biphasic and poorly differentiated depending upon the cells present. Monophasic variety is composed entirely of spindle cells and the biphasic variety consists of both spindle cells and the epithelial cells. [3] Majority of patients with PPSS are young adults between the age group of 30 and 50 years. [3] Females and males have equal incidence with no sex predilection. [4] Typical signs and symptoms include chest pain, dyspnea, cough and hemoptysis. In a large series reported by Zeren et al., up to 40% of patients were asymptomatic and PPSS was an incidental finding on chest radiograph. [5] Fever, chest heaviness and shoulder pain are additional symptoms that occur less frequently. [5] Most common presentation is of a well-circumscribed mass with effusion on CT, although recurrent hemothorax and pneumothorax have been reported as the initial manifestation of the disease. [1] Because of the rarity of pleural synovial sarcoma and its similarity (clinical and histologic) to other pleural neoplasms, particularly sarcomatous mesothelioma, diagnosis is often difficult. Presence of Bcl-2 protein represents a useful marker for differentiating synovial sarcoma, especially monophasic variants from mesothelioma. [6] In our case Bcl-2 positivity favored the diagnosis of synovial sarcoma. The differential diagnosis of a spindle cell neoplasm in the pleural cavity includes sarcomatoid malignant mesothelioma, solitary fibrous tumor, hemangiopericytoma and sarcomatoid carcinoma. [7] Rare pleural tumors include pleural primary effusion lymphoma, which is associated with human herpes virus-8 (HHV-8) and Epstein-Barr virus (EBV), [8] desmoplastic small round cell tumor, pleuropulmonary blastoma, and other sarcomas like liposarcoma and vascular sarcoma. [9] Immunohistochemistry and cytogenetic analysis are useful in confirming the diagnosis. [3] Immunohistochemically, most synovial sarcomas display at least focal immunoreactivity for cytokeratin and epithelial membrane antigen, which is usually more prominent in the epithelial component. MIC-2 and Bcl-2 are also detected in the majority of cases. Synovial sarcomas are negative for CD34. [6] The chromosomal translocation t(X;18)(p11.2;q11.2) has been found in more than 90% of synovial sarcomas, regardless of the histologic subtype. This translocation results in the fusion of SYT gene on chromosome 18 to either the SSX1 or SSX2 gene on chromosome X. Several techniques can be used to identify the translocation or fusion transcript, including conventional cytogenetic analysis, molecular cytogenetic analysis (FISH) and reverse transcriptase-polymerase chain reaction assay. The best treatment for synovial sarcomas of the pleura remains unclear. Its treatment is similar to that of synovial sarcomas occurring at other sites. [10] A multidisciplinary approach, including surgery, chemotherapy and radiotherapy, has been suggested. Surgery (radical resection) is the first treatment of synovial sarcoma. Adjuvant radiotherapy is usually recommended after incomplete resection. [11] The benefits of chemotherapy are unclear, however, improvement in survival has been described with doxorubicin and ifosfamide. [12] Neoadjuvant chemotherapy could be an appropriate option to allow the radical surgical procedure by reducing tumor volume and potentially treating micrometastatic disease. Radiotherapy, chemotherapy and radiofrequency thermal ablation may be considered as alternative treatments for inoperable patients.

 > Conclusion Top

Primary pleural synovial sarcoma is a rare neoplasm of pleura. It can present as multiple pleural nodules with associated pleural effusion. Though rare, it should be considered in the differential diagnosis of hemorrhagic pleural effusion in a young adult.

 > References Top

1.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. 1
2.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 1996;20:36-45.  Back to cited text no. 2
3.Tailor J, Roy PG, Bowker C, Lakhoo K. Primary pleural synovial sarcoma presenting as a multiloculated cyst in an adolescent. Pediatr Surg Int 2008;24:597-9.  Back to cited text no. 3
4.Mirzoyan M, Muslimani A, Setrakian S, Swedeh M, Daw HA. Primary pleuropulmonary synovial sarcoma. Clin Lung Cancer 2008;9:257-61.  Back to cited text no. 4
5.Zeren H, Moran CA, Suster S, Fishback NF, Koss MN. Primary pulmonary sarcomas with features of monophasic synovial sarcoma: A clinicopathological, immunohistochemical, and ultrastructural study of 25 cases. Hum Pathol 1995;26:474-80.  Back to cited text no. 5
6.Nicholson AG, Goldstraw P, Fisher C. Synovial sarcoma of the pleura and its differentiation from other primary pleural tumours: A clinicopathological and immunohistochemical review of three cases. Histopathology 1998;33:508-13.  Back to cited text no. 6
7.Cappello F, Barnes L. Synovial sarcoma and malignant mesothelioma of the pleura: Review, differential diagnosis and possible role of apoptosis. Pathology 2001;33:142-8.  Back to cited text no. 7
8.Melo NC, Sales MM, Santana AN, Costalonga EC, Pedreira AB, Ianhez LE. Pleural primary effusion lymphoma in a renal transplant recipient. Am J Transplant 2008;8:906-7.  Back to cited text no. 8
9.Granville L, Laga AC, Allen TC, Dishop M, Roggli VL, Churg A, et al. Review and update of uncommon primary pleural tumors: A practical approach to diagnosis. Arch Pathol Lab Med 2005;129:1428-43.  Back to cited text no. 9
10.Loscertales J, Triviño A, Gallardo G, Congregado M. Primary monophasic synovial sarcoma of the pleura: Diagnosis and treatment. Interact Cardiovasc Thorac Surg 2011;12:885-7.  Back to cited text no. 10
11.Eilber FC, Dry SM. Diagnosis and management of synovial sarcoma. J Surg Oncol 2008;97:314-20.  Back to cited text no. 11
12.Silva RG, Gross JL, Silva RA, Haddad FJ, Younes RN, Cruz VM, et al. Primary monophasic synovial sarcoma of the pleura: neoadjuvant chemotherapy followed by complete resection. Thoracic Cancer 2010;1:95-101.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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