Synovial Cell Sarcoma Workup
- Author: Bernardo Vargas, MD; Chief Editor: Harris Gellman, MD more...
Plain radiographs may aid in the diagnosis. Synovial sarcoma typically produces a spotty calcification (snowstorm) within the matrix of the soft tissue tumor that may be visualized on plain radiographs (see the image below).
Computed tomography (CT) is used to confirm the presence of a mass, its size, and its location, but it is nondiagnostic. It also may detect secondary bony involvement, which is not uncommon with larger synovial sarcomas. Synovial sarcoma is a malignant disease; therefore, CT of the chest is mandatory to exclude metastatic disease.
Magnetic resonance imaging
Magnetic resonance imaging (MRI) is the investigation of choice for soft-tissue sarcomas. Low signal intensity is observed on T1-weighted MRI (see the first image below), and high signal intensity is observed on T2-weighted MRI (see the second image below). The signal from the matrix is fairly homogeneous unless calcification is present.
Cytogenetic analysis aids the physician in detecting the specific chromosomal translocation between chromosome 18 and chromosome X.[16, 18, 19] This translocation produces the SYT-SSX1, SYT-SSX2, or SYT-SSX4 fusion gene. Identification of this anomaly is obtained by RT-PCR in tumor tissue, with a sensitivity of 96% and specificity of 100%. Fusion transcripts can be detected with molecular diagnostic techniques in biopsy samples.
Fluorescence in situ hybridization (FISH) is less expensive than RT-PCR. It has been suggested as a good method of first choice. This method has a lower sensitivity than RT-PCR does, but the results of the two methods are concordant in 76% of cases.
The histologic picture of synovial cell carcinoma can be confused with those of many other round blue cell tumors. Macroscopically, the tumor is a grayish-white and often has a greasy feel. Histologic features of synovial sarcoma are identical in children and adults.
Three types of synovial sarcoma have been described:
In the monophasic type, there is a predominance of spindle cells, mixed with round cells (see the image below); cells are arranged in fascicles with a poorly defined cytoplasm; no glandular areas are present
The biphasic type has a layer of columnar epithelium in addition to spindle cells; it consists of plump, round cells and spindle-shaped fibroblasts alternating with glandular-like areas that are lined by synovial-like cells and contain mucin
The histologic grade is determined by a score of the mitotic activity and tumor necrosis, ranging from grade 1 (differentiated) to grade 3 (poorly differentiated). Synovial sarcoma is very often associated with a grade 2 or grade 3.[6, 9, 10, 20] Punctuate areas of calcification may be observed.
Staging requires local imaging with MRI. The most likely site of distant spread, the lungs, can be observed by means of CT. Synovial sarcoma is a high-grade lesion.
Cytogenetic analysis aids the physician in detecting the specific chromosomal translocation t(X;18)(p11;q11). The chromosomal translocation produces either the SYT-SSX1 or the SYT-SSX2 fusion gene, which can be identified by reverse transcriptase-polymerase chain reaction (RT-PCR) in tumor tissue and possibly in blood.[10, 11, 17]
Aside from molecular diagnostic tests, no laboratory studies are specific for this diagnosis.
Eilber FC, Dry SM. Diagnosis and management of synovial sarcoma. J Surg Oncol. 2008 Mar 15. 97(4):314-20. [Medline].
Spira AI, Ettinger DS. The use of chemotherapy in soft-tissue sarcomas. Oncologist. 2002. 7(4):348-59. [Medline].
Siehl JM, Thiel E, Schmittel A, Hütter G, Deckert PM, Szelényi H, et al. Ifosfamide/liposomal daunorubicin is a well tolerated and active first-line chemotherapy regimen in advanced soft tissue sarcoma: results of a phase II study. Cancer. 2005 Aug 1. 104(3):611-7. [Medline].
Zagars GK, Ballo MT, Pisters PW, Pollock RE, Patel SR, Benjamin RS, et al. Prognostic factors for patients with localized soft-tissue sarcoma treated with conservation surgery and radiation therapy: an analysis of 1225 patients. Cancer. 2003 May 15. 97(10):2530-43. [Medline].
Ladanyi M, Antonescu CR, Leung DH, Woodruff JM, Kawai A, Healey JH, et al. Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients. Cancer Res. 2002 Jan 1. 62(1):135-40. [Medline].
Deshmukh R, Mankin HJ, Singer S. Synovial sarcoma: the importance of size and location for survival. Clin Orthop Relat Res. 2004 Feb. 155-61. [Medline].
Ladenstein R, Treuner J, Koscielniak E, d'Oleire F, Keim M, Gadner H, et al. Synovial sarcoma of childhood and adolescence. Report of the German CWS-81 study. Cancer. 1993 Jun 1. 71(11):3647-55. [Medline].
Knösel T, Heretsch S, Altendorf-Hofmann A, Richter P, Katenkamp K, Katenkamp D, et al. TLE1 is a robust diagnostic biomarker for synovial sarcomas and correlates with t(X;18): analysis of 319 cases. Eur J Cancer. 2010 Apr. 46(6):1170-6. [Medline].
Antonescu CR, Kawai A, Leung DH, Lonardo F, Woodruff JM, Healey JH, et al. Strong association of SYT-SSX fusion type and morphologic epithelial differentiation in synovial sarcoma. Diagn Mol Pathol. 2000 Mar. 9(1):1-8. [Medline].
Guillou L, Coindre J, Gallagher G, Terrier P, Gebhard S, de Saint Aubain Somerhausen N, et al. Detection of the synovial sarcoma translocation t(X;18) (SYT;SSX) in paraffin-embedded tissues using reverse transcriptase-polymerase chain reaction: a reliable and powerful diagnostic tool for pathologists. A molecular analysis of 221 mesenchymal tumors fixed in different fixatives. Hum Pathol. 2001 Jan. 32(1):105-12. [Medline].
Kawai A, Woodruff J, Healey JH, Brennan MF, Antonescu CR, Ladanyi M. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med. 1998 Jan 15. 338(3):153-60. [Medline].
Kawaguchi S, Wada T, Ida K, Sato Y, Nagoya S, Tsukahara T, et al. Phase I vaccination trial of SYT-SSX junction peptide in patients with disseminated synovial sarcoma. J Transl Med. 2005 Jan 12. 3(1):1. [Medline]. [Full Text].
Thompson RC Jr, Garg A, Goswitz J, Cheng EY, Clohisy DR, Dusenbery K. Synovial sarcoma. Large size predicts poor outcome. Clin Orthop Relat Res. 2000 Apr. 18-24. [Medline].
Al-Daraji W, Lasota J, Foss R, Miettinen M. Synovial sarcoma involving the head: analysis of 36 cases with predilection to the parotid and temporal regions. Am J Surg Pathol. 2009 Oct. 33(10):1494-503. [Medline].
Bridge JA. Cytogenetic and molecular cytogenetic techniques in orthopaedic surgery. J Bone Joint Surg Am. Apr 1993. 75(4):606-14. [Medline].
Ten Heuvel SE, Hoekstra HJ, Suurmeijer AJ. Diagnostic accuracy of FISH and RT-PCR in 50 routinely processed synovial sarcomas. Appl Immunohistochem Mol Morphol. 2008 May. 16(3):246-50. [Medline].
Kind M, Stock N, Coindre JM. Histology and imaging of soft tissue sarcomas. Eur J Radiol. 2009 Oct. 72(1):6-15. [Medline].
Lessnick SL, Dei Tos AP, Sorensen PH, Dileo P, Baker LH, Ferrari S, et al. Small round cell sarcomas. Semin Oncol. 2009 Aug. 36(4):338-46. [Medline].
Bergh P, Meis-Kindblom JM, Gherlinzoni F, Berlin O, Bacchini P, Bertoni F, et al. Synovial sarcoma: identification of low and high risk groups. Cancer. 1999 Jun 15. 85(12):2596-607. [Medline].
Fukukawa C, Hanaoka H, Nagayama S, Tsunoda T, Toguchida J, Endo K, et al. Radioimmunotherapy of human synovial sarcoma using a monoclonal antibody against FZD10. Cancer Sci. 2008 Feb. 99(2):432-40. [Medline].
Sakabe T, Murata H, Konishi E, Takeshita H, Ueda H, Matsui T, et al. Evaluation of clinical outcomes and prognostic factors for synovial sarcoma arising from the extremities. Med Sci Monit. 2008 Jun. 14(6):CR305-310. [Medline].
Mazeron JJ, Suit HD. Lymph nodes as sites of metastases from sarcomas of soft tissue. Cancer. 1987 Oct 15. 60(8):1800-8. [Medline].
Ghert MA, Abudu A, Driver N, Davis AM, Griffin AM, Pearce D, et al. The indications for and the prognostic significance of amputation as the primary surgical procedure for localized soft tissue sarcoma of the extremity. Ann Surg Oncol. 2005 Jan. 12(1):10-7. [Medline].
Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol. 1996 Mar. 14(3):859-68. [Medline].
Moreno Martín-Retortillo L, Andrés Moreno MM, Cañete Nieto A, Castel Sánchez V. Synovial sarcoma in children. A single centre experience. Clin Transl Oncol. 2007 Jul. 9(7):468-70. [Medline].
Stefanovski PD, Bidoli E, De Paoli A, Buonadonna A, Boz G, Libra M, et al. Prognostic factors in soft tissue sarcomas: a study of 395 patients. Eur J Surg Oncol. 2002 Mar. 28(2):153-64. [Medline].
Spillane AJ, A'Hern R, Judson IR, Fisher C, Thomas JM. Synovial sarcoma: a clinicopathologic, staging, and prognostic assessment. J Clin Oncol. 2000 Nov 15. 18(22):3794-803. [Medline].
Campbell C, Gallagher J, Dickinson I. Synovial sarcoma--towards a simplified approach to prognosis. ANZ J Surg. 2004 Sep. 74(9):727-31. [Medline].
Crowson MG, Lalich I, Keeney MG, Garcia JJ, Price DL. Clinicopathologic factors and adjuvant treatment effects on survival in adult head and neck synovial cell sarcoma. Head Neck. 2014 Jan 15. [Medline].