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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 607-608
Synovial sarcoma of thigh


Department of Radiodiagnosis, Dyanand Medical College & Hospital, Ludhiana - 141001, India

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Date of Submission21-Aug-2006
Date of Acceptance10-Nov-2006
 

Keywords: Sarcoma, Synovial

How to cite this article:
Kalia V, Saggar K, Sandhu P, Ahluwalia A, Vibhuti. Synovial sarcoma of thigh. Indian J Radiol Imaging 2006;16:607-8

How to cite this URL:
Kalia V, Saggar K, Sandhu P, Ahluwalia A, Vibhuti. Synovial sarcoma of thigh. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:607-8. Available from: http://www.ijri.org/text.asp?2006/16/4/607/32280
Synovial Sarcomas are rare fibroblastic soft tissue neoplasms that account for 7-8% of all human malignant sarcomas. Although this tumor generally affects adults, about 30-50% of reported cases occur in children and adolescents. The usual clinical manifestations are painless slow growing extremity mass. In the appropriate clinical setting, the diagnosis can be suggested on imaging, however, the final diagnosis comes from tissue biopsy. Prompt diagnosis is necessary as delay in the diagnosis is related to poor prognosis. This case report describes a case of synovial sarcoma of the thigh, a rare pediatric tumor of the extremities.

A 11 year old female child presented with a progressively increasing swelling in left upper thigh of 10 months duration in February 2006. The swelling was hard on palpation, tenderness was present and overlying skin was normal. She complaints of restriction of movement at the hip joint however, no systematic symptoms were present. Suspecting a soft tissue pathology, plain radiograph of the left thigh was done which revealed a soft tissue mass in the left upper thigh however, underlying bones appear normal. Areas of calcification were seen within the mass [Figure - 1]. Following this, the patient underwent ultrasound and computed tomography (CT) examination of left thigh. Sonography showed a lobulated solid mass with calcification and vascularity within it. Small hypoechoic areas suggestive of cystic degeneration were also present. Contrast enhanced CT of left thigh revealed large lobulated heterogeneous soft tissue density mass with extensive calcification occupying the anterior compartment of the left thigh. It is extending from the level of roof of acetabulum for a distance of approximately 9.7 centimeters inferiorly and measures 10.1 X 6.6 centimeters. On giving intravenous contrast, significant enhancement was seen. Overlying skin is thickened with indistinct fat planes with iliopsoas anal adductor longus. Superficial femoral vessels were encased by this mass however no intraluminal thrombus was seen. Underlying bones were normal [Figure - 2]. Based on CT findings, the possibility of malignant soft tissue tumor was given and patient was operated. On histopathological examination, biphasic pattern of cells forming cystic/gland like structures, moderate nuclear pleomorphism and spindle shaped cells arranged as interlacing bands were seen. At places, there was myxoid material, foci of calcification and cystic spaces. The findings were diagnostic of Synovial Sarcoma of the left thigh.


   Discussion Top


Synovial Sarcoma is the third most common pediatric soft tissue tumor and most common non-rhabdomyosarcomatous soft tissue sarcoma in pediatric patients. Despite its name it does not appear to arise from the synovial membrane but rather from unknown multipotent stem cells that are capable of differentiating into mesenchymal and/or epithelial structures [1]. Various synonyms for this tumor are synovial endothelioma, synovioma, synovial sarcoma, synovialoma, synovial sacroendothelioma, mesothelioma and sarcomesothelioma [2].

Etiology of this neoplasm remains unknown. It may arise "de novo" from undifferentiated mesenchymal cells and almost all cells are characterized by the presence of a translocation involving chromosomes X and 18 [t(X;18)(P11.2;q11.2)]. This translocation is specific to synovial sarcoma and constitute an excellent tool to diagnose this malignancy[1]. Approximately 85% of synovial sarcomas arise in the extremities. Other sites of its origin are abdominal wall, head and neck, lungs, oesophagus, intestine, mediastinum, retroperitoneum and pelvis (precoccygeal) [1],[2],[3],[4]. Nonetheless, most lesions are extremity tumors that are para articular in location and may arise from tendon sheaths, bursae or joint capsules but seldom involve the articular surface [1],[4]. In pediatric age groups, male : female ratio is 1:1.6 and girls clearly outnumber the boys. The most common presenting symptom is a painless mass of several weeks to years duration. Four other patterns of presentation also exist: a pretumor phase of pain and tenderness without a mass, acute arthritis or bursitis, a chronic contracture, and a tumor noted after an episode of trauma [1],[3]. On imaging, approximately 30% of the synovial sarcomas reveal calcification that may be apparent on Plain films or CT Scan [3]. On CT, the tumor shows heterogenous multilocated appearance with varying degrees of internal septations. On magnetic resonance imaging, they tend to be sharply marginated tumors that may reveal triple signal intensity of fluid, fat and fibrous tissue. Occasionally, fluid-fluid levels can be seen in them.

Most of the tumors were intimately related to bone and can show cortical thinning or medullary invasion [5],[6]. They commonly metastatise to lung (74-81%), lymph nodes (12-23%) and bone (20%). Chest CT is used to detect pulmonary metastases, and 99 technetium labelled methyldiphosphonate nuclear bone scanning is used to detect bone metastatic disease [2],[3]. Histopathologically, they were classified into 4 types:

a) Biphasic synovial sarcoma

b) Monophasic fibrous synovial sarcoma

c) Monophasic epithelial synovial sarcoma

d) Poorly differentiated synovial sarcoma

Most of the tumors have predominantly biphasic pattern followed by monophasic pattern and then by poorly differentiated pattern. The optimal approach to the treatment of synovial sarcoma remains undefined because no prospective clinical trials have compared different therapeutic approaches [7],[8]. Complete surgical resection of the primary tumor is the mainstay of treatment. Adjuvant radiotherapy to treat microscopic residual disease after surgery provides excellent control and obviates amputation for most patients. The role of adjuvant chemotherapy remains controversial and most regimens that have shown activity in patients with measurable disease include doxorubicin with or without an alkylating agent (cyclophosphamide or ifosfamide). Prognosis of the tumor depend on various clinical and pathological features e.g. features associated with adverse outcome are increasing age, tumor size 5cms, lower extremity location, presence of poorly differentiated areas, particularly when atleast 20% of the tumor have grade 3 nuclei, rhabdoid cells and more than 10 mitotic figures / 10 High power fields [9]. Imaging findings favouring a high grade tumor include proximal distribution, large tumor size > 10cms, absence of calcification, tumor possessing cyst; presence of hemorrhage and triple signal pattern [10].

 
   References Top

1.Ruggiero A. Synovial Sarcoma. Orphanet Encyclopedia. March 2004 http://www.orpha.net/data/patho/GB/uk-synovialsarcoma.pdf  Back to cited text no. 1    
2.Cadman NL, Soule EH, Kelly PJ. Synovial Sarcoma. An analysis of 134 tumors. Cancer 1965; 613-627.  Back to cited text no. 2    
3.McCarville MB, Spunt SL, Skapek SK, Pappo AS. Synovial Sarcoma in Pediatric Patients. Am. J. of Roentgenol 2002; 179: 797-801.  Back to cited text no. 3    
4.Coffin CM, Synovial based tumors and Synovial Sarcoma. In: Coffin CM, O'Shea PA, Dehner LP, eds Pediatric soft tissue tumors: a clinical pathological and therapeutic approach, 1st ed. Baltimore: Williams & Wilkins, 1997: 295-310.  Back to cited text no. 4    
5.Lee SM, Hajdu SI, Exelby PR. Synovial Sarcoma in children. Surg. Gynecol Obstet. 1974; 138: 701-704.  Back to cited text no. 5    
6.Jones BC, Sundaram M, Karensdorf MJ. Synovial sarcoma. MR imaging findings in 34 patients. Am. J. of Roentgenol 1993; 161:827-830.  Back to cited text no. 6    
7.Miser JS, Triche TJ, Kinsella TJ, Pritchard DJ. Other soft tissue sarcomas of childhood. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology, 3rd Ed. Philadelphia: Lippincott -Raven, 1997: 865-888.  Back to cited text no. 7    
8.Brennan MF, Alektiar KM, Maki RG. Sarcomas of the soft tissues and bones. In: De Vita VT Jr, Hellman S, Rosenberg SA, eds. Cancer: principles and practice of Oncology, 6th ed. Philadelphia: Lippincott Williams and Wilkins, 2001: 1841-1891.  Back to cited text no. 8    
9.Machen SK, Easley KA, Goldblum JR. Synovial sarcoma of the extremities: a clinicopathological study of 34 cases, including semi-quantitative analysis of spindled, epithelial and poorly differentiated areas. Am. J. Surg. Pathol. 1999; 23(3): 268-275.  Back to cited text no. 9    
10.Tateishi H, Hasegawa T, Beppu Y, Satahe M, Moriyama N. Synovial sarcoma of the soft tissues: prognostic significance of imaging features. J. Comput. Assist. Tomog. 2004; 28(1): 140-148.  Back to cited text no. 10    

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Correspondence Address:
V Kalia
464-Sec 8/Ii, Urban Estate , Karnal, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32280

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