A 63-year-old woman presenting with a synovial sarcoma of the hand: a case report

Journal of Medical Case Reports

Maria Manuel Mouzinho¹  Maria Manuel Mendes⁵  Carlos Mavioso⁵  Conceição Azeda⁵  Manuela Mafra⁴  Ana Isabel Ribeiro²  Diogo Casal³ 

[1] Plastic and Reconstructive Surgery Department, Hospital de São José, Rua José António Serrano, 1150-199, Lisbon, Portugal;Pathology Resident, São José Hospital, Lisbon, Portugal;Plastic and Reconstructive Surgery Resident, São José Hospital, Lisbon, Portugal;Pathology Senior Consultant, São José Hospital, Lisbon, Portugal;Plastic and Reconstructive Surgery Senior Consultant, São José Hospital, Lisbon, Portugal

关键词: Malignant tumor;    Surgery;    Hand;    Synovial sarcoma;   

Others  :  1195256
DOI  :  10.1186/1752-1947-6-385

 received in 2012-06-04, accepted in 2012-10-05,  发布年份 2012

【 摘 要 】

Introduction

Synovial sarcoma is a high-grade, soft-tissue sarcoma that most frequently is located in the vicinity of joints, tendons or bursae, although it can also be found in extra-articular locations. Most patients with synovial sarcoma of the hand are young and have a poor prognosis, as these tumors are locally aggressive and are associated with a relatively high metastasis rate. According to the literature, local recurrence and/or metastatic disease is found in nearly 80% of patients. Current therapy comprises surgery, systemic and limb perfusion chemotherapy, and radiotherapy. However, the 5-year survival rate is estimated to be only around 27% to 55%. Moreover, most authors agree that synovial sarcoma is one of the most commonly misdiagnosed malignancies of soft tissues because of their slow growing pattern, benign radiographic appearance, ability to change size, and the fact that they may elicit pain similar to that caused by common trauma.

Case presentation

We describe an unusual case of a large synovial sarcoma of the hand in a 63-year-old Caucasian woman followed for 12 years by a multidisciplinary team. In addition, a literature review of the most pertinent aspects of the epidemiology, diagnosis, treatment and prognosis of these patients is presented.

Conclusion

Awareness of this rare tumor by anyone dealing with hand pathology can hasten diagnosis, and this, in turn, can potentially increase survival. Therefore, a high index of suspicion for this disease should be kept in mind, particularly when evaluating young people, as they are the most commonly affected group.

【 授权许可】

   
2012 Casal et al.; licensee BioMed Central Ltd.

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【 参考文献 】

• [1]Murray PM: Soft tissue sarcoma of the upper extremity. Hand Clin 2004, 20:325-333. vii
• [2]Athanasian EA: Bone and soft tissue tumors. In Green’s Operative Surgery. Volume 2. 6th edition. Edited by Wolfe SW, Hotchkiss RN, Pederson WC, Kozin SH. Philadelphia: Churchill Livingstone; 2011:2141-2191.
• [3]Siegel HJ, Sessions W, Casillas MA Jr, Said-Al-Naief N, Lander PH, Lopez-Ben R: Synovial sarcoma: clinicopathologic features, treatment, and prognosis. Orthopedics 2007, 30:1020-1027.
• [4]Andrassy RJ, Okcu MF, Despa S, Raney RB: Synovial sarcoma in children: surgical lessons from a single institution and review of the literature. J Am Coll Surg 2001, 192:305-313.
• [5]Deshmukh R, Mankin HJ, Singer S: Synovial sarcoma: the importance of size and location for survival. Clin Orthop Relat Res 2004, 419:155-161.
• [6]Kampe CE, Rosen G, Eilber F, Eckardt J, Lowenbraun S, Foster J, Forscher C, Selch M: Synovial sarcoma. A study of intensive chemotherapy in 14 patients with localized disease. Cancer 1993, 72:2161-2169.
• [7]Weitz J, Antonescu CR, Brennan MF: Localized extremity soft tissue sarcoma: improved knowledge with unchanged survival over time. J Clin Oncol 2003, 21:2719-2725.
• [8]Sultan I, Rodriguez-Galindo C, Saab R, Yasir S, Casanova M, Ferrari A: Comparing children and adults with synovial sarcoma in the Surveillance, Epidemiology, and End Results program, 1983 to 2005: an analysis of 1268 patients. Cancer 2009, 115:3537-3547.
• [9]De Silva MV, Barrett A, Reid R: Premonitory pain preceding swelling: a distinctive clinical presentation of synovial sarcoma which may prompt early detection. Sarcoma 2003, 7:131-135.
• [10]Wong CH, Chow L, Yen CH, Ho PC, Yip R, Hung LK: Uncommon hand tumours. Hand Surg 2001, 6:67-80.
• [11]Nakajima H, Matsushita K, Shimizu H, Isomi T, Nakano Y, Saito M, Aoki H: Synovial sarcoma of the hand. Skeletal Radiol 1997, 26:674-676.
• [12]Ferrari A, Gronchi A, Casanova M, Meazza C, Gandola L, Collini P, Lozza L, Bertulli R, Olmi P, Casali PG: Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution. Cancer 2004, 101:627-634.
• [13]Miettinen M, Virtanen I: Synovial sarcoma: a misnomer. Am J Pathol 1984, 117:18-25.
• [14]Beck AH, West RB, van de Rijn M: Gene expression profiling for the investigation of soft tissue sarcoma pathogenesis and the identification of diagnostic, prognostic, and predictive biomarkers. Virchows Arch 2010, 456:141-151.
• [15]Shi W, Indelicato DJ, Morris CG, Scarborough MT, Gibbs CP, Zlotecki RA: Long-term treatment outcomes for patients with synovial sarcoma: a 40-year experience at the University of Florida. Am J Clin Oncol 2012.
• [16]Wolden SL, Alektiar KM: Sarcomas across the age spectrum. Semin Radiat Oncol 2010, 20:45-51.
• [17]Eilber FC, Dry SM: Diagnosis and management of synovial sarcoma. J Surg Oncol 2008, 97:314-320.
• [18]Okcu MF, Despa S, Choroszy M, Berrak SG, Cangir A, Jaffe N, Raney RB: Synovial sarcoma in children and adolescents: thirty three years of experience with multimodal therapy. Med Pediatr Oncol 2001, 37:90-96.
• [19]Talbot SG, Mehrara BJ, Disa JJ, Wong AK, Pusic A, Cordeiro PG, Athanasian EA: Soft-tissue coverage of the hand following sarcoma resection. Plast Reconstr Surg 2008, 121:534-543.