ARTICLE IN PRESS doi:10.1510/icvts.2010.259531 Editorial
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Case report - Thoracic oncologic
Department of General Thoracic Surgery, Virgen Macarena University Hospital, Seville, Spain Received 3 November 2010; received in revised form 14 January 2011; accepted 19 January 2011
Proposal for Bailout Procedure
Keywords: Sarcoma; Synovial; Pleura; Treatment
1. Case report
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Fig. 1. (a) Chest X-ray: large mass visible at right lung base. (b) CT-scan: heterogeneous mass adhered to right hemidiaphragm, and compressing mediastinal structures.
Brief Case Report Communication
䊚 2011 Published by European Association for Cardio-Thoracic Surgery
Negative Results
A 68-year-old female presented with cough and severe dyspnea. A chest X-ray (Fig. 1a) and computed tomography (CT)-scan (Fig. 1b) revealed a large tumor measuring 15=13 cm at the right lung base. A transthoracic needle biopsy was performed, and the biopsy specimen revealed a spindle cell tumor. Posterolateral thoracotomy enabled clear visualization and identification of a large encapsulated mass in the right hemidiaphragm, adhering to and infiltrating the middle lobe. Since the tumor could not be dissected from this lobe, a middle lobectomy was performed; the diaphragm from which the tumor arose was also extirpated since separation by radical surgery was impossible. The diaphragm defect was repaired using a 7=5 cm Gore-Tex䊛 (W.L. Gore & associates INC, Newark, NJ, USA) Dual-Mesh patch (2 mm thickness). Clinical and radiological progress was good, and the patient was discharged on the fifth postoperative day. Definitive pathologic tumor size was 15.5=13=10 cm, and weighed 920 g. Grossly, it was whitish-gray in color, well-defined, and friable; papillary and hemorrhagic areas were observed. At histopathological examination (Fig. 2a), the neoplasm was identified as a malignant mesenchymal spindle-cell tumor, with a mitotic index of 25y10 HPF. Immunohistochemical staining (Fig. 2b,c) revealed positivity for B-cell lymphoma 2 (Bcl-2), and focally for CK7, CK19, EMA, CD99; tumor cells stained negative for S-100 protein, calretinin, Ckit, HMB-45, AML, CD34, EDA and desmin. Cytogenetic analysis and FISH (Fig. 2d) identified *Corresponding author. Av. Dr Fedriani 3, 41071 Seville, Spain. Tel.: q34955-008206; fax: q34-955-008205. E-mail address:
[email protected] (A. Trivin ˜o).
ESCVS Article
Synovial sarcomas are uncommon soft tissue malignancies that usually affect the extremities in the vicinity of large joints. The recognition of this tumor in an unexpected site, such as the pleura, is often difficult and the monophasic variant of synovial sarcoma is often mistaken for some other spindle cell tumor. In this report, we describe a very rare case of primary monophasic synovial sarcoma of the pleura treated with radical surgery. 䊚 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.
Institutional Report
Abstract
Protocol
Jesus Loscertales, Ana Trivin ˜o*, Gregorio Gallardo, Miguel Congregado
Work in Progress Report
Primary monophasic synovial sarcoma of the pleura: diagnosis and treatment
New Ideas
Interactive CardioVascular and Thoracic Surgery 12 (2011) 885–887
ARTICLE IN PRESS 886
J. Loscertales et al. / Interactive CardioVascular and Thoracic Surgery 12 (2011) 885–887
Fig. 2. (a) Hematoxylin and eosin-stained section shows spindle cells arranged in fascicles. Tumor cells show focal positivity for CK7 (b) and strong positivity for Bcl-2 (c). (d) FISH analysis reveals the chromosomal translocation (X;18). Bcl-2, B-cell lymphoma 2.
a translocation (X;18). These results served to rule out melanoma, malignant neurogenic tumors, mesothelioma and carcinoma, and supported our diagnosis. 2. Discussion Synovial sarcoma is a rare type of soft tissue tumor w1x. Nowadays near 36 case have been reported since Gaertner et al. published the first case in 1996 w2x. Differential diagnosis of synovial sarcoma is very difficult because of its rarity and its quite similar clinical and histologic characteristics to other spindle-cell pleural tumor, mainly with sarcomatous mesothelioma. Inmunohistochemical findings to identify synovial sarcoma from other pleural neoplasms include positive cytokeratin staining and epithelial membrane antigen w3, 4x. Positive finding of bcl2 antibodies is used to exclude mesothelioma (usually negative w4x). Cytogenetic studies are useful for the differential diagnosis. Chromosomal translocation (X;18) is found in over 90% of synovial sarcomas and can be detected by the FISH method (fluorescence in-situ hybridization) w5x. In spite of its high sensitivity, the FISH method is not obligatory for the diagnosis, and it is indicated in cases of histological and inmunohistochemical overlapping w5x, as in our case. The best treatment for synovial sarcomas of the pleura remains unclear. Because a synovial sarcomas of the pleura is rare, the paucity of data regarding its natural history and the limited number of published cases, its treatment is similar to other localization synovial sarcoma’s treatment. A multidisciplinary approach, including surgery, che-
motherapy and radiotherapy has been suggested w6x. Surgery (radical resection) is the first treatment of synovial sarcoma. Adjuvant radiotherapy is usually recommended after incomplete resection or after extensive resection of large tumors w6, 7x. The benefits of chemotherapy are unclear; however, improvement in survival has been described with doxorubicin and ifosfamide w3x. In our case, complete resection was performed, but the patient refused adjuvant therapy. So unfortunately the CT-scans showed recurrence five months later. Neoadjuvant chemotherapy could be an appropriate option to allow the radical surgical procedure by reducing tumor volume and potentially treating micrometastasis disease w3x. Radiotherapy, chemotherapy and radiofrequency thermal ablation may be considered as alternative treatments for inoperable patients w8x. Other options have been reported, Abe et al. w9x suggest combined hyperthermia therapy with chemoradiotherapy for the patient with advanced inoperable primary pleural synovial sarcoma. Synovial sarcoma is an aggressive tumor with a very poor prognosis compared to other sarcomas. Recurrences are common and patients may require multiple surgical resections. A five-year disease-free period of 20.9% w10x has been reported. In conclusion, there is no gold standard therapy because synovial sarcoma is a rare malignancy with a difficult differential diagnosis and not enough data are available due to the few cases that have been quoted in the literature.
ARTICLE IN PRESS J. Loscertales et al. / Interactive CardioVascular and Thoracic Surgery 12 (2011) 885–887
M, Tanaka Y, Uematsu A, Sugitani M. Radiological assessment following thermoradiation therapy for primary pleural synovial sarcoma: case report. Med Oncol 2010;27:1027–1030.
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w10x Be ´gueret H, Galateau-Salle F, Guillou L, Chetaille B, Brambilla E, Vignaud JM, Terrier P, Groussard O, Coindre JM. Primary intrathoracic synovial sarcoma: a clinicopathologic study of 40 t(X;18) – positive cases from the French Sarcoma Group and the Mesopath Group. Am J Surg Pathol 2005;29:339–346.
Work in Progress Report
Surg Oncol 2008;97:314–320. w8x Lee HK, Kwon HJ, Lee HB, Jin GY, Chung MJ, Lee YC. Radiofrequency thermal ablation of primary pleural synovial sarcoma. Respiration 2006;73:250–252. w9x Abe K, Maebayashi T, Shizukuishi T, Sakaguchi M, Furuhashi S, Takahashi
New Ideas
w1x Taylor J, Roy PG, Bowker C, Lakhoo K. Primary pleural synovial sarcoma presenting as a multiloculated cyst in an adolescent. Pediatr Surg Int 2008;24:597–599. w2x Gaertner E, Zeren H, Fleming MV, Colby TV, Travis WD. Biphasic synovial sarcomas arising in the pleural cavity: a clinicopathologic study of five cases. Am J Surg Pathol 1996;20:36–45. w3x Silva RGM, Gross JL, Silva RA, Haddad FJ, Younes RN, Cruz VM, AvertanoRocha ABM. Primary monophasic synovial sarcoma of the pleura: neoadjuvant chemotherapy followed by complete resection. Thoracic Cancer 2010;1:95–101. w4x Vohra HA, Davies S, Vohra H, Rosin MD, Snead DR. Primary synovial sarcoma of the pleura: beware of misdiagnosis. Eur Intern Med 2004;15:465–466. w5x Aubry MC, Bridge JA, Wickert R, Tazelaar HD. Primary monophasic synovial sarcoma of the pleura: five cases confirmed by the presence of SYT-SSX fusion transcript. Am J Surg Pathol 2001;25:776–781.
w6x Carson JH, Harwood AR, Cummings BJ, Fornasier V, Langer F, Quirt I. The place of radiotherapy in the treatment of synovial sarcoma. Int J Radiat Oncol Biol Phys 1981;7:49–53. w7x Eilber FC, Dry SM. Diagnosis and management of synovial sarcoma. J
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References
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