Abstract Primary cardiac synovial sarcoma is an extremely rare disease. Its prognosis is poor with a median survival time of 24 months, even when adequate and timely treatment is given. This case reports a 61-year-old woman presenting with primary cardiac synovial sarcoma with an 8-year survival time following surgical and adjuvant chemotherapy. Cardiac synovial sarcoma, Cardiac tumor, Cardiac mass resection INTRODUCTION Primary cardiac cancers are rare, with prevalence in autopsy series estimated between 0.001% and 0.03% . About 1 quarter of these are malignant . Sarcomas represent 3 quarters of these malignant lesions, and 4.2% of sarcomas are primary cardiac synovial sarcomas (PCSSs) . Prognosis is poor with a median survival time of 24 months , even when proper treatment—complete surgical excision in association with both radiotherapy and chemotherapy—is conducted. We report a rare case of an 8-year survival time in a patient presenting with synovial sarcoma. CASE REPORT In August 2009, a 61-year-old Caucasian woman was referred to our institution for dyspnoea on exertion [New York Heart Association (NYHA) III], lipothymia and lower chest pain that had started 1 month earlier. Medical history was remarkable for a breast adenoma. A transthoracic echocardiography (TTE) detected pulmonary artery dilatation, obstacle subvalvular ejection and a moderate pulmonary insufficiency. A thoracic computed tomography (T-CT) scan revealed an atypical myocardial mass in the pulmonary infundibulum with extension to the intraluminal pulmonary trunk. There were no signs of distal embolism or abnormality of the left heart (Fig. 1). The magnetic resonance imaging (MRI) identified a mass suggesting an angiosarcoma. The 1st workup for extension was carried out using a positron emission tomography scan, which showed a vascularized mass with an intense hyperfixation of the right ventricle, without any secondary localization. Figure 1: View largeDownload slide (A and B) A computed tomography scan image of the lesion, (C) anatomical specimen and (D) histological preparation. Figure 1: View largeDownload slide (A and B) A computed tomography scan image of the lesion, (C) anatomical specimen and (D) histological preparation. Because of clinical status and imaging results, the patient was finally determined suitable for a surgical treatment. The complete resection of the mass was conducted en bloc, and the reconstruction was performed with a Goretex patch for the right ventricle wall and a biological valved tube implantation (Medtronic Freestyle® valve No. 27) for the pulmonary tract. A postoperative histological study displayed a high-grade synovial sarcoma (Fig. 1). The genetic study was positive for SYT/SSX1, thus confirming the diagnosis. Adjuvant chemotherapy using doxorubicin–ifosfamide was performed for 6 months, achieving a complete eradication of the disease. The follow-up control was performed twice per year. Seven years later, the routine TTE control detected a 20-mm round-shaped lesion in the pulmonary infundibulum, suggestive of a neoplastic recurrence. A positron emission tomography scan was performed, revealing a moderate fixation of the right ventricular mass and a hyperfixation of a subaortic lymph node. The patient underwent a 2nd operation for the removal of the mass (Fig. 2). Recovery was uneventful. Thereafter, and following directions from the oncological team, the patient benefitted from an adjuvant 6-month cyclophosphamide chemotherapy cycle. During the 12-month follow-up, the patient showed neither clinical nor imaging signs of recurrence, and the patient is currently asymptomatic. Figure 2: View largeDownload slide (A) Anatomical specimen and (B) histological preparation. Figure 2: View largeDownload slide (A) Anatomical specimen and (B) histological preparation. DISCUSSION Although extremely rare, PCSSs are high-grade tumours. The diagnosis must always be obtained using TTE and T-CT or MRI to characterize the mass before a surgical intervention. In fact, these lesions are often misdiagnosed with a myxoma, leading to an incomplete surgical resection. As reported in various articles, most of these lesions undergo surgery only after TTE, and the correct PCSS diagnosis is only obtained at the time of the postoperative histological examination. In any case, the ‘gold standard’ diagnostic of PCSS is histological and must be conducted using fluorescence in situ hybridization or real-time polymerase chain reaction. A reciprocal translocation between SYT gene on chromosome 18 and SSX1 gene on chromosome X is a specific cytogenetic abnormality that occurs consistently in these lesions . The complete surgical excision remains the only treatment to significantly improve the survival rate. Several authors have reported different chemotherapy and radiotherapy protocols, but there is no evidence of their efficiency  thus far. In our case, the preoperative diagnosis based on TTE, T-CT, MRI and positron emission tomography has led to a more precise preoperative characterization of the mass, thereby ensuring the complete excision of such mass. Following histological confirmation of PCSS, we performed an adjuvant chemotherapy using doxorubicin–ifosfamide over 6 months. This strategy has led to the longest survival time described in the literature so far. Even when we reported a recurrence of 7 years following the primary intervention, timely surgery and adjuvant 6-month cyclophosphamide chemotherapy have permitted a total recovery during the 12-month follow-up. The aggressiveness of PCSS requires a regular follow-up, which we advise performing twice a year to detect a recurrence in time. CONCLUSION In conclusion, when a cardiac mass is detected on TTE, we strongly recommend performing T-CT or MRI prior to surgery to better assess the nature of the lesion. We underline the need for a radical and early surgical excision. We suggest adjuvant doxorubicin–ifosfamide chemotherapy, despite its efficiency still yet to be proven, along with an intensive follow-up twice per year. Conflict of interest: none declared. REFERENCES 1 Centofanti P, Di Rosa E, Deorsola L, Dato GM, Patanè F, La Torre M et al. Primary cardiac tumors: early and late results of surgical treatment in 91 patients. Ann Thorac Surg 1999; 68: 1236– 41. Google Scholar CrossRef Search ADS PubMed 2 Vander Salm TJ. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg 2000; 12: 89– 100. Google Scholar CrossRef Search ADS PubMed 3 Wang J-G, Li N-N. Primary cardiac synovial sarcoma. Ann Thorac Surg 2013; 95: 2202– 9. Google Scholar CrossRef Search ADS PubMed 4 Putnam JBJr, Sweeney MS, Colon R, Lanza LA et al. Primary cardiac sarcomas. Ann Thorac Surg 1991; 51: 906– 10. Google Scholar CrossRef Search ADS PubMed 5 Varma T, Adegboyega P. Primary cardiac synovial sarcoma. Arch Pathol Lab Med 2012; 136: 454– 8. Google Scholar CrossRef Search ADS PubMed © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)
Interactive CardioVascular and Thoracic Surgery – Oxford University Press
Published: Mar 30, 2018
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