Ann Thorac Surg 2004;78:e41-e42
© 2004 The Society of Thoracic Surgeons
Case report
Pericardial synovial sarcoma: 14-year survival with multimodality therapy
Gerry Van der Mieren, MDa,
Stefan Willems, MDb,
Raf Sciot, MD, PhDb,
Herlinde Dumez, MDc,
Allan Van Oosterom, MD, PhDc,
Willem Flameng, MD, PhDa,
Paul Herijgers, MD, PhDa,*
a Department of Cardiac Surgery, Leuven, Belgium
b Department of Pathology, Leuven, Belgium
c Department of Medical Oncology, Katholieke Universiteit Leuven, Leuven, Belgium
Accepted for publication February 3, 2004.
* Address reprint requests to Dr Herijgers, Department of Cardiac Surgery, K. U. Leuven, U. Z. Herestraat 49, Leuven B-3000, Belgium
paul.herijgers{at}med.kuleuven.ac.be
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Abstract
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We report a case of recurrent primary synovial sarcoma of the pericardium. Reverse transcriptase-polymerase chain reaction analysis for t(X,18) demonstrated the presence of the chimeric transcript SYT/SSX. Because of the rarity of this entity, optimal therapy is unknown. The prognosis of this tumor is very poor in previous reports. In this report, we present a case with five recurrences treated by a combination of surgery, chemotherapy, and radiotherapy. The patient survives now for more than 14 years, the longest reported survival of a primary synovial sarcoma of the pericardium.
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Introduction
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Primary synovial sarcoma of the pericardium is extremely rare, with only a few case reports available [1–4]. The prognosis of patients with this tumor is very poor with a disease-free survival of 12 months after surgery [1], an overall survival of 7 months [2], and survival of 4 months after surgery with residual tumor [3]. We present a case of recurrent primary pericardial synovial sarcoma with a survival of 14 years.
A 26-year-old man presented in July 1989 with signs of cardiac tamponade. Chest roentgenogram and computed tomographic (CT) scan revealed a mediastinal mass, superior of the right ventricle, expanding between the ascending aorta and vena cava superior, reaching up to 2 cm above the carina. The mass arising from the pericardial reflection near the ascending aorta was resected through a median sternotomy. Histologic examination revealed a biphasic pattern with epithelial and spindle cell components (Fig 1). Mitotic figures were occasionally encountered (low grade). Immunohistochemical analysis showed expression of vimentin, cytokeratins, and epithelial membrane antigen. The diagnosis of biphasic, low grade synovial sarcoma was established.
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Fig 1. Photomicrograph of a typical biphasic appearance of a biphasic synovial sarcoma. The plump epithelial cells have ovoid nuclei and form glandular structures and solid nests. The spindle cells have sparse cytoplasm, short fusiform, or small nuclei. They have indistinct cell borders and form intersecting fascicles between the glandular structures. (Hematoxylin and eosin, x40.)
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During yearly clinical follow-ups, no signs of tumor activity were found until December 1996. The patient then complained of fatigue and retrosternal pain during forced inspiration. Computed tomographic scan and magnetic resonance imaging showed a 35 x 40 mm mass between the vena cava superior and the ascending aorta (Fig 2). A biopsy performed through parasternotomy at a regional hospital was comparable with the specimen of 1989. A second resection by median sternotomy was performed. Resection margins were microscopically negative. No adjuvant radiotherapy or chemotherapy was performed. Immunohistochemical analysis with avidin-biotin immunoperoxidase techniques, using antibodies for cytokeratins, epithelial membrane antigen, and epidermal growth factor receptor (EGFR) was positive for the epithelial component of the tumor cells. Cytogenetic analysis for t(X,18) by demonstration of SYT/SSX transcripts by reverse transcriptase-polymerase chain reaction was positive.
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Fig 2. Magnetic resonance image from December 1996 shows the first tumor recurrence between the vena cava superior and the aorta.
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In May 1998, a new CT scan demonstrated a mass of 24 x 23 mm, which was found ventral of the vena cava superior, ascending aorta, and right atrium, without signs of metastases by further investigations. After four cycles of adriamycine (75 mg/m2 every 3 weeks) with reduction of the mass to 10 mm, a resection of the residual tumor was performed. Histologic analysis revealed a necrotic nodule with a few islands of biphasic pericardial synovial sarcoma in the border. Resection margins were microscopically negative.
In June 1999, a third recurrence was detected with CT scan and was localized left of the aortic arch. Positron-emission tomographic scan revealed no metastases. Two cycles of ifosfamide (3 g/m2/d on 3 subsequent days, 3-week intervals) had no effect. The mass was resected through median sternotomy. Microscopic examination showed recurrence of the synovial sarcoma with a mitosis index of 37/10 high-power field. Resection margins were negative.
In January 2002, a mass of 49 x 33 mm was found to be left and superior of the aortic arch. Four cycles of ifosfamide (3 g/m2/d on 3 subsequent days, 3-week intervals) and adriamycine (20 mg/m2, 3 times every 3 weeks) caused a subtotal remission to 34 x 19 mm. The residual tumor was excised through partial superior sternotomy. Microscopic findings showed a recurrence with a mitosis index of 4/200 high-power field with positive resection margins. Therefore, radiotherapy 60 Gray in 30 fractions was delivered, without residual tumor afterward on CT scan.
In January 2003, a CT scan demonstrated the recurrence of a small mediastinal mass ventral of the vena cava superior. Magnetic resonance imaging and positron-emission tomographic scan could not differentiate between recurrence of tumor or postradiotherapy sequel. In March 2003, the CT scan showed expansion of the mass to 33 x 33 x 45 mm. A second nodule of 13 mm in the left costodiaphragmatic sinus was found, which was interpreted as a pleural metastasis. The tumor cells in the previous recurrence showed an EGFR expression of 70%. Therefore, induction therapy with gefitinib (an EGFR-tyrosine kinase inhibitor) (500 mg/d) was started. In May 2003, the CT scan showed further enlargement of both known tumor locations, and multiple new intraparenchymatous pulmonary lesions. A palliative therapy with doxorubicinehydrochloride (40 mg/m2 every 4 weeks) chemotherapy was stopped after two cycles because of disease progression. Subsequently, ecteinascidin 743 (Yondelis) was started in July 2003, but was stopped 3 months later because of disease progression. The patient refused further therapy.
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Comment
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Primary synovial sarcoma of the pericardium is extremely rare. In only four of the case reports, synovial sarcoma from cardiac tissue has the pericardium as its primary origin. Because of its rarity, little is known about survival, and adjuvant therapy is not standardized [1].
Broad surgical resection is the cornerstone of therapy. In this case, as in other reports, the presenting sign was cardiac tamponade, necessitating an urgent operation. A disease-free survival of 7 years was obtained after the first broad resection with negative resection margins. Radiotherapy is recommended with positive resection margins [1], as was the case after the fourth recurrence in our patient.
The place for chemotherapy of this tumor is not well-defined, and in none of the other case reports [1–4], preoperative chemotherapy was given. In our patient, the best response was obtained with adriamycine and the combination of adriamycine and ifosfamide. Ifosfamide as monotherapy yielded no response, and there was tumor growth while the patient was treated with gefitinib, doxorubicinehydrochloride, and ecteinascidin 743. The place for chemotherapy of extremity soft tissue and retroperitoneal sarcomas is largely dependent on size, grade, and location. Anthracyclines and ifosfamide have been established as the most active chemotherapeutic agents. Several new molecular targets are analyzed [5]. We would recommend induction chemotherapy with adriamycin or adriamycin and ifosfamide depending of the age and performance status of the patient, when broad resection seems as though it is not possible because of tumor growth close to vital structures, or in the case of recurrence. Furthermore, chemotherapy may have its place when surgical resection is impossible due to the extension of the primary tumor or metastases.
All recurrences were detected with clinical follow-up (first) or CT scan (all others). Therefore, CT or magnetic resonance imaging should be performed every 6 months to detect subclinical recurrences, to be able to perform a new broad resection in the absence of metastases, and to prolong survival as much as possible.
We report that a long and symptom-free survival of this terminal disease can be obtained with an aggressive multimodality therapy and intensive follow-up. Our patient survived 14 years, which we believe is the longest survival of this disease.
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References
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- Al-Rajhi N, Husain S, Coupland R, et al. Primary pericardial synovial sarcoma: a case report and literature review. J Surg Oncol. 1999;70:194–198[Medline]
- Oizumi S, Igarashi K, Takenaka T, et al. Primary pericardial synovial sarcoma with detection of the chimeric transcript SYT-SSX. Jpn Circ J. 1999;63:330–332[Medline]
- Langner K, Schäfer R, Müller KM, et al. Synovial sarcoma of the pericardium. Pathologe. 1998;19:442–446[Medline]
- Kojima KY, Koslin DB, Primack SL. Synovial sarcoma arising from the pericardium: radiographic and CT findings. Am J Roentgenol. 1999;173:246–247[Medline]
- Borden EC, Baker LH, Bell RS, et al. Soft tissue sarcomas of adults: state of the translational science. Clin Cancer Res. 2003;9:1941–1956[Abstract/Free Full Text]
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Abstract
Introduction
