Journal of Clinical Oncology, Vol 18, Issue 11
(June), 2000: 2341-2342
© 2000 American Society for Clinical Oncology
Two Patients With Sarcoma
Arthur Skarin, MDConsultant Editor
M. Sekeres,
M. J. Vasconcelles,
M. Mcmenamin,
M. Rosenfeld-Darling,
R. Bueno
Dana-Farber Cancer Institute, Brigham and Women’s Hospital, and Harvard Medical School, Boston, MA
CASE 1. SYNOVIAL CELL SARCOMA OF THE LUNG
A 35-year-old woman developed increasing pleuritic, right-sided back pain. Other symptoms included dyspnea on exertion, a cough productive of bloody sputum, a low-grade temperature associated with rigors, and a sore throat. An uncle developed lung cancer at the age of 60 years. Physical examination revealed clear lungs and no lymphadenopathy. Laboratory examination showed a WBC count of 22.6 x 109/L with 83% neutrophils and 11% bands, a lactate dehydrogenase level of 176 U/L (normal range, 107 to 231 U/L), and an erythrocyte sedimentation rate of 35 mm/h (normal range, 0 to 20 mm/h). A chest radiograph showed a large, ovoid mass in the proximity of the major fissure on the right, and a computed tomography scan of the chest verified an 8 x 7 x 6.5 cm mass within or abutting the major fissure without any associated lymphadenopathy (Fig 1). A fine-needle biopsy of the mass was nondiagnostic. Because of increasing hemoptysis, the mass, along with the right-lower and right-middle lobes of the lung, was resected. Gross appearance showed considerable congestion and areas of hemorrhage (Fig 2). Histopathology revealed sheets of small, round cells with scant cytoplasm and focal areas of spindle cells with abundant mitoses (Fig 3). The tumor displayed immunohistochemical staining that was strongly positive focally for keratins (Fig 4) and diffusely positive for O-13 and MIC-2, with negative staining for the S-100 protein. Cytogenetics indicated a translocation between chromosomes X and 18, and a diagnosis of synovial cell sarcoma was made. The tumor was removed with clean margins, but washings of the pleural cavity revealed positive cytology. Postoperatively, the patient initiated adjuvant doxorubicin and ifosfamide and is doing well.
Synovial sarcoma is the third most common histologic type of extremity soft tissue sarcoma, preceeded by liposarcoma and malignant fibrous histiocytoma.1 Although it rarely involves the lung as a primary site of disease, the incidence of primary intrathoracic tumors may be increasing because of growing awareness and diagnosis of this clinicopathologic entity.2 Synovial sarcoma is associated with the t(X;18)(p11;q11), resulting in either the SYT-SSX1 or SYT-SSX2 fusion genes and subsequent transcripts. Some evidence suggests that the particular fusion transcript generated by the translocation correlates with both the histologic subtype and the clinical behavior of the disease.3,4 Further, this translocation can be helpful in distinguishing primary synovial cell sarcoma of the lung from other small-cell tumors with similar histology and immunohistochemical staining,5 particularly primary neuroectodermal tumor, which is typified by a t(11;22). Prognostic factors for survival in primary synovial sarcoma include primary tumor size (< 5 cm v > 10 cm), margin of resection (clean margin v microscopic positive), and mean mitotic activity (< 10 mitoses per 10 high-power fields v > 10 mitoses per 10 high-power fields), with overall cancer-specific survival for localized synovial sarcoma reaching 34% at 10 years.6 Therapy for localized resectable soft tissue sarcoma of adults involves resection followed by consideration of adjuvant doxorubicin-based chemotherapy, which has been shown in a meta-analysis to reduce the risk of local recurrence by 27% and the risk of distant metastases by 30% but to have no definitive impact on overall survival.7
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