Ann Thorac Surg 2008;85:2120-2122. doi:10.1016/j.athoracsur.2008.01.004
© 2008 The Society of Thoracic Surgeons
Case Reports
Primary Synovial Sarcoma of the Posterior Chest Wall
Jung-Jyh Hung, MDa,b,
Teh-Ying Chou, MD, PhDc,
Chih-Hao Sun, MDc,
Jung-Sen Liu, MD, PhDa,
Wen-Hu Hsu, MDd,*
a Department of Surgery, Cathay General Hospital, School of Medicine, Fu Jen Catholic University, Taipei, Taiwan
b Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan
c Department of Pathology, Taipei, Taiwan
d Division of Thoracic Surgery, Department of Surgery, Taipei Veterans General Hospital and School of Medicine, National Yang-Ming University, Taipei, Taiwan
Accepted for publication January 2, 2008.
* Address correspondence to Dr Hsu, Division of Thoracic Surgery, Department of Surgery, Taipei Veterans General Hospital, No 201, Sec 2, Shih-Pai Rd, Taipei, 112, Taiwan (Email: whhsu{at}vghtpe.gov.tw).
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Abstract
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Synovial sarcoma is a malignant soft-tissue tumor that most commonly occurs in the extremities of young adults. Only several cases of synovial sarcomas of the chest wall and pleura had been reported. We present a 24-year-old man who had right back pain, chest pain, dyspnea, and intermittent fever from a huge primary synovial sarcoma of the right posterior chest wall. Multimodality therapies, including surgical resection, and chemotherapy and radiation therapy were applied, but the tumor progressed rapidly and the patient died 6 months after diagnosis. Prompt diagnosis and aggressive surgical resection is mandatory for primary synovial sarcoma of the chest wall because of its aggressive behavior.
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Introduction
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Synovial sarcomas comprise approximately 5% to 10% of all soft tissue sarcomas. Primary synovial sarcoma of the chest wall is rare [1–3]. We present the case of a young patient presenting with right back pain, chest pain, dyspnea, and intermittent fever, and a primary synovial sarcoma in the right posterior chest wall. Aggressive local recurrence and pulmonary metastasis of the synovial sarcoma occurred soon after palliative resection during follow-up.
A 24-year-old man was admitted to the thoracic surgery department because of right back pain for 6 months, and nonproductive cough, chest pain, shortness of breath, and intermittent fever for 1 month. Physical examination revealed no abnormal physical finding except for decreased breath sounds on the right. The chest roentgenogram revealed total opacity of the right hemithorax. Computed tomography (CT) of the chest demonstrated a heterogeneously enhanced mass (18 x 15 x 12 cm) in the posterior mediastinum, with compression of the heart, esophagus, inferior vena cava, and right pulmonary parenchyma (Fig 1A). Right 10th rib destruction and massive right pleural effusion were also revealed. A CT-guided biopsy revealed synovial sarcoma. A whole body bone scan revealed no evidence of bony metastasis except increased uptake in the 9th and 10th costovertebral junction.
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Fig 1. (A) Computed tomography (CT) of the chest shows a heterogeneously enhanced mass (18 x 15 x 12 cm) in the posterior mediastinum with compression to the heart, esophagus, inferior vena cava, and right pulmonary parenchyma. (B) A CT of the chest performed 5 weeks postoperatively shows a huge mass at right posterior mediastinum, with progressive change compared with preoperative images.
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The patient underwent surgical resection through a right posterolateral thoracotomy. Intraoperatively, a 20- x 20- x 12-cm, macroscopically yellowish, soft mass arising from soft tissue of the ninth intercostal space near the costovertebral junction was found. The tumor invaded the 10th thoracic vertebra, right lower lobe of the lung, pericardium, mediastinum, and diaphragm. Palliative resection of the tumor with right lower lobe lobectomy was done, with residual tumor in ninth intercostal space near the costovertebral junction.
Microscopic examination of the specimen showed monophasic synovial sarcoma composed of spindle cells with fibroblast-like appearance and focally whorled pattern (Fig 2A). The tumor cells showed immunoreactivity for Bcl-2 and vimentin but negative for CK (AE1/AE3) and epithelial membrane antigen (Fig 2B).
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Fig 2. (A) Histopathologic examination shows monophasic synovial sarcoma composed of spindle cells with fibroblast-like appearance and focally whorled pattern (hematoxylin-eosin stain, original magnification x100). (B) The tumor cells showed immunoreactivity for Bcl-2 (Bcl-2 stain, original magnification x400).
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The patient was discharged in stable condition and received adjuvant radiotherapy 4 weeks after the operation. However, a postoperative CT of the chest at 5 weeks revealed a huge mass in the right posterior mediastinum and progressive change compared with preoperative images, with adjacent bony destruction and pulmonary metastasis (Fig 1B). The patient received additional chemotherapy with epirubicin and ifosfamide thereafter. Unfortunately, the response was poor, and the tumor still progressed. Multiple bone metastases occurred 4.5 months postoperatively. He died 6 months after diagnosis.
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Comment
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Synovial sarcoma most commonly occurs in the extremities of young adults, typically in a juxtaarticular location. Primary synovial sarcoma of the thorax is rare [1, 4]. It may arise within the mediastinum, heart, lung, pleura, or chest wall. Primary synovial sarcomas of the chest wall are extremely rare [1–3]. The common symptoms of synovial sarcoma of the chest include dyspnea, chest pain, cough, and hemoptysis [4].
Although synovial sarcoma is frequently associated with joints, tendons, and bursal structures, it does not arise from the synovium [5]. The histogenesis of synovial sarcomas is not well known, but the tumors may be derived from pluripotential mesenchymal cells capable of epithelial differentiation [5]. The specific chromosomal translocation t(X;18) (p11.2;q11.2), found in more than 90% of all synovial sarcomas, serves as a marker for this tumor. At CT, a synovial sarcoma that arises in the chest wall is characterized most commonly as a heterogeneously enhanced mass with well-defined margins, cortical bone destruction, tumor calcifications, and tumor infiltration of the chest wall musculature [1, 4]. Mediastinal, hilar, diaphragmatic or axillary lymph nodes are rarely involved [1]. In the current case, preoperative CT of the chest revealed massive pleural effusion.
The prognosis of synovial sarcoma is generally modest, with a 5-year overall survival rate of 50% to 80%, depending on the patient's age, the tumor size, and its resectability [6]. Multimodality therapy for synovial sarcomas includes surgical resection, chemotherapy, and radiation therapy [6]. Radiation therapy is an adjuvant treatment for local control of synovial sarcoma and may be used in preoperative and postoperative settings [7]. Synovial sarcoma has moderate chemosensitivity, with about 50% response rates to regimens containing ifosfamide and doxorubicin [7]. When the tumor involves extremities, the presentation may be much earlier because of pain and the presence of a lump or mass, and treatment may be more effective because of the option of amputation. In the chest, presentation may be delayed, and the tumor may be advanced before a diagnosis is made.
The study by Duran-Mendicuti and colleagues [1] showed a high recurrence rate of primary synovial sarcoma of the chest in a short interval of 2 to 14 months after surgical resection. All patients with primary synovial sarcoma of the chest in their report were treated by resection and were alive at 9 to 58 months of follow-up [1].
Although the recurrence rate is high, surgical resection should be pursued when synovial sarcoma occurs within the chest. In the present patient, palliative resection was performed for the huge synovial sarcoma arising from posterior chest wall with pleuropulmonary and mediastinal involvement. Aggressive local recurrence and pulmonary metastasis occurred soon after palliative resection during follow-up. The size and extent of the residual tumor at 5 weeks postoperatively were even larger than those before the operation. Chemotherapy and radiotherapy had little therapeutic effect in the current case. Mukhopadhyay and Aubry [3] reported a similar case of recurrent primary synovial sarcoma of the anterior chest wall.
Because of its aggressive behavior, differential diagnosis of chest masses in young adults should include synovial sarcoma for prompt diagnosis and management.
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References
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- Duran-Mendicuti A, Costello P, Vargas SO. Primary synovial sarcoma of the chest: radiographic and clinicopathologic correlation J Thorac Imaging 2003;18:87-93.[Medline]
- Bui-Mansfield LT, Kaplan KJ, Boardman J. Radiologic-pathologic conference of Keller Army Community Hospital at West Point, the United States Military Academy: synovial sarcoma of the chest wall AJR Am J Roentgenol 2002;179:880.[Free Full Text]
- Mukhopadhyay S, Aubry MC. Recurrent primary synovial sarcoma of the chest wall J Thorac Oncol 2007;2:660-661.[Medline]
- Frazier AA, Franks TJ, Pugatch RD, Galvin JR. From the archives of the AFIP: Pleuropulmonary synovial sarcoma Radiographics 2006;26:923-940.[Abstract/Free Full Text]
- Miettinen M, Virtanen I. Synovial sarcoma–a misnomer Am J Pathol 1984;117:18-25.[Abstract]
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- Wolden SL. Radiation therapy for non-rhabdomyosarcoma soft tissue sarcomas in adolescents and young adults J Pediatr Hematol Oncol 2005;27:212-214.[Medline]
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Abstract
Introduction
