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J Thorac Cardiovasc Surg 2007;134:808-809
© 2007 The American Association for Thoracic Surgery
Brief Communication |
a Division of Thoracic Surgery, European Institute of Oncology, Milan, Italy
b Division of Pathology and Laboratory Medicine, European Institute of Oncology, Milan, Italy
c University of Milan School of Medicine, Milan, Italy.
Received for publication May 22, 2007; accepted for publication May 31, 2007. * Address for reprints: Domenico Galetta, MD, Division of Thoracic Surgery, European Institute of Oncology, Via Ripamonti 435, 20141, Milan, Italy. (Email: mimgaletta{at}yahoo.it).
Synovial sarcoma is a malignant neoplasm predominantly affecting soft tissues of the extremities in adolescents and young adults and might rarely occur in other anatomic locations. The lung, mediastinum, pleura, and chest wall have also recently been found to be sites affected primarily by this sarcoma.1-3
We evaluated the clinical and pathologic features of primary synovial sarcoma (PSS) of the chest and the role of surgical intervention and tried to identify possible prognostic factors affecting survival.
Between October 1998 and December 2006, 15 patients (7 men) underwent a resection of PSSs of the chest, which were categorized into pulmonary, mediastinal, and chest wall PSSs according to the tumor location. Medical records of these patients were analyzed for age, sex, preoperative symptoms, radiologic findings, pathology, surgical procedures, clinical outcome, and long-term survival.
All pathologic slides were rereviewed, and PSSs were confirmed by means of immunohistochemical and fluorescence in situ hybridization studies. Fluorescence in situ hybridization analysis was performed for the identification of the diagnostic chromosomal translocation (t[X;18][p11.2;q11.2]), a marker for this tumor, resulting from fusion of the SYT gene on chromosome 18 to either the SSX1 or SSX2 gene on chromosome X.
Median age was 52 years (range, 21–77 years). Computed tomographic scans of the chest (Figure 1 and Figure E1) were performed in all patients and demonstrated a pulmonary mass in 8 patients, a mediastinal neoplasm in 2 patients (anterior and posterior, respectively), and a chest wall tumor in 5 patients. A positron emission tomographic scan was performed in 4 patients, showing high tumor activity in all of them (maximal standardized uptake value: range, 4.5–11.2; median, 6.6).
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Tumor size ranged from 3.5 to 21 cm (median, 8 cm). Six tumors were larger than 10 cm. Macroscopically, in 10 cases tumors presented as well-circumscribed masses. Histopathologic diagnosis confirmed a biphasic tumor in 1 patient (Figure E1, A and inset), monophasic fibrous tumors in 13 patients (Figure E1, B), and a poorly differentiated tumor in 1 patient (Figure E1, C). Thirteen patients showed diffuse immunoreactivity for epithelial membrane antigen and keratins. Bcl-2 and S-100 results were negative in all patients. The tumor was positive for t(X;18) in all patients, confirming the diagnosis of synovial sarcoma (Figure E2, C, inset).
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Median follow-up was 25 months (range, 1-148 months). Local recurrence developed in 7 (46.6%) patients, 2 (13.3%) of whom had distant metastasis. The 5-year disease-free interval rate was 30% (median, 15 months). The 10-year survival rate was 33.5%. Six (40%) patients are currently alive, 3 (20%) with disease (overall median survival, 27 months).
Factors that adversely affected survival include a tumor dimension of greater than 10 cm (P = .0062), incomplete resection (P = .0114), and no adjuvant therapy (P = .035).
PSSs involving the thorax are rare entities reported in the literature either as single observations or small series.1-4 They usually occur in adults, and the mean age at diagnosis is 38 years.2 Our series showed that thoracic PSS is diagnosed at an average age of 52 years and showed no sex predilection. Our findings are in keeping with prior studies and emphasize that PSS of the chest belongs in the differential diagnosis of chest masses in young adults (localized fibrous tumors of the pleura, mesothelioma, fibrosarcoma, and metastatic disease).
Immunohistochemical findings that distinguish synovial sarcoma from other sarcomas include positive staining for cytokeratin and EMA. Synovial sarcomas lack staining for S-100 and smooth muscle markers.
Optimal treatment of PSS of the chest has not been defined. Multimodal therapy of surgical intervention, chemotherapy, and radiotherapy has been used. Recurrences are likely and might be treated with further resection when possible or with radiotherapy. In our series chemotherapy and radiotherapy helped to control the recurrences, with a long disease-free survival.
In conclusion, thoracic PSS is a very uncommon and aggressive malignancy with poor prognosis. The best treatment is not yet defined. Complete resection is feasible and leads to an acceptable local control and long-term survival.
References
This article has been cited by other articles:
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  G. Pelosi, A. Sonzogni, T. De Pas, D. Galetta, G. Veronesi, L. Spaggiari, M. Manzotti, C. Fumagalli, E. Bresaola, O. Nappi, et al. Review Article: Pulmonary Sarcomatoid Carcinomas: A Practical Overview International Journal of Surgical Pathology, April 1, 2010; 18(2): 103 - 120. [Abstract] [PDF]
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