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Cancer Medicine: Case Discussion Primary Pulmonary Synovial Sarcoma: A Case Report and Review of Current Diagnostic and Therapeutic Standards SHERI DENNISON, ERIC WEPPLER, GEORGE GIACOPPE Department of Internal Medicine, Madigan Army Medical Center, Tacoma, Washington, USA Key Words. Sarcoma · Malignancy · Pleura · Synovial · Genetics
L EARNING O BJECTIVES After completing this course, the reader will be able to: 1. Describe primary pulmonary synovial sarcoma as a rare tumor that usually arises in young adults as a large pleuralbased intrathoracic mass. 2. Explain that the diagnosis is made by histology, immunohistochemical studies, and, more recently, by identification of the SYT-SSX1 or SYT-SSX2 chromosomal translocations. 3. List factors that portend a worse prognosis, including inability to achieve a complete resection, larger tumor size, male sex, older age, high-grade tumor with necrosis, and the SYT-SSX1 variant. CME
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A BSTRACT A 30-year-old female presented with hemoptysis, chest pain, and a rapidly enlarging pleural-based mass, and was found to have primary synovial sarcoma of the lung. Primary pulmonary sarcomas comprise 5 cm), male gender, older age (>20 years), extensive tumor necrosis, high grade, large number of mitotic figures (>10 per 10 high-powered fields), neurovascular invasion, and, recently, the SYT-SSX1 variant [6]. The main prognostic factor is the ability to achieve a complete resection. There is no standardized therapy; most patients are treated with surgery or with surgery and adjuvant radiation therapy. The rarity of this tumor has not permitted controlled studies of adjuvant chemotherapy. Synovial sarcomas are chemosensitive to ifosfamide and doxorubicin, with an overall response rate of approximately 24% [7]. In a meta-analysis, adjuvant chemotherapy for sarcomas improved the time to local recurrence and recurrence-free survival rate, with a trend toward a better overall survival rate.
R EFERENCES 1 Keel SB, Bacha E, Mark EJ et al. Primary pulmonary sarcoma: a clinicopathologic study of 26 cases. Mod Pathol 1999;12:1124-1131.
2 Gaertner E, Zeren EJ, Fleming MV et al. Biphasic synovial sarcomas arising in the pleural cavity: a clinicopathologic study of five cases. Am J Surg Pathol 1996;20:36-45.
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3 Weiss S, Goldblum J. Enzinger and Weiss’s Soft Tissue Tumors, Fourth Edition. St. Louis, MO: Mosby, 2001:1502-1504.
eration and poor clinical outcome. Cancer Res 1999;59:31803184.
4 Kawai A, Woodruff J, Healey JH et al. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med 1998;338:153-160.
6 Trassard M, Le Doussal V, Hacene K et al. Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients. J Clin Oncol 2001;19:525-534.
5 Nilsson G, Skytting B, Xie Y et al. The SYT-SSX1 variant of synovial sarcoma is associated with a high rate of tumor cell prolif-
7 Spillane AJ, A’Hern R, Judson IR et al. Synovial sarcoma: a clinicopathologic, staging, and prognostic assessment. J Clin Oncol 2000;18:3794-3803.
