Successful robotic resection of a primary cardiac synovial sarcoma.
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ABSTRACT:
SUBMITTER: Akhmerov A
PROVIDER: S-EPMC8298838 | biostudies-literature | 2020 Jun
REPOSITORIES: biostudies-literature
Publications
Successful robotic resection of a primary cardiac synovial sarcoma.
Akhmerov Akbarshakh A Chung Joshua S JS Emerson Dominic D Chikwe Joanna J Trento Alfredo A
JTCVS techniques 20200224
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Project description:A 48-year-old man presented with several months of dyspnea and edema. He had no medical history. Transthoracic echocardiography showed a large mass in the tricuspid orifice. Enhanced computed tomography revealed that the mass had extended outside the heart by breaking though the right atrium wall. Hemodynamics has not collapsed yet, but his symptoms were rapidly worsening. He underwent emergency surgery, and complete resection was achieved. The pathologic diagnosis was primary cardiac synovial sarcoma. There were no signs of recurrence or metastasis 6 months after the surgery without additional treatment. Cardiac tumors are rare and develop silently, so they can be easily missed. Computed tomography and echocardiography provide much information quickly and help us to comprehend general condition. This case highlights their efficiency and the importance of early detection.
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Project description:Primary cardiac synovial sarcomas are very rare, representing <1% of all primary cardiac tumors. We report the case of a 19-year-old man with syncope and dynamic obstructive shock caused by a large right-sided intracardiac tumor. (Level of Difficulty: Beginner .).
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Project description:ObjectiveTo evaluate the outcomes of surgical resection of malignant primary cardiovascular tumors.MethodsFrom 1983 to 2018, 32 patients underwent surgical resection of malignant primary cardiovascular sarcoma at Cleveland Clinic. Mean age was 48 ± 15 years, and 19 (59%) were women. Outcomes are compared between those with complete resection and those without, and in relation to primary location.ResultsThe most common histologic subtypes were angiosarcoma (n = 8 [25%]) and high-grade undifferentiated sarcoma (n = 7 [22%]). Fourteen (44%) involved the left heart, 9 (28%) the right heart, 8 (25%) the pulmonary arteries, and 1 (3%) the aorta. There was clinical evidence of isolated extracardiac metastases in 8 (25%). Six (19%) patients were deemed unresectable at surgery, undergoing biopsy and palliative debulking followed by referral for definitive chemotherapy and/or radiation. The remaining 26 (81%) patients underwent 31 tumor resections with curative intent. Seven (22%) patients had previously undergone a resection or biopsy at another institution. There were 10 second-time resections, 2 third-time resections, 1 fourth-time resection, and no operative mortalities. Median survival was 3 years, with estimated survival at 6 months and 1, 5, and 10 years of 90%, 73%, 31%, and 17%, respectively. Of the 8 (25%) who were considered disease-free following surgery, 4 experienced recurrences during follow-up.ConclusionsPrimary cardiac sarcoma continues to be a challenging disease with poor prognosis. Aggressive resection with curative intent, frequent surveillance for local and distant recurrence, and systemic and local multimodality treatment optimizes outcomes.
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Project description:ObjectivePrimary pulmonary synovial sarcoma (PPSS) is extremely rare. This study aims to identify the clinicopathologic and therapeutic factors determining survival in PPSS.MethodsWe performed a retrospective analysis of 121 patients from the Surveillance, Epidemiology, and End Results Database as well as 12 patients from our own institution diagnosed with PPSS. Patient survival was evaluated using the Kaplan-Meier method.ResultsThe median survival time for 12 PPSS patients in our institution was 78 months. Postoperative chemotherapy (P = .027 for overall survival and P = .035 for disease-specific survival) was associated with superior survival, whereas pneumonectomy (P = .011 for overall survival and P = .006 for disease-specific survival) was associated with worse survival. Single lobe involvement (P = .022) and the absence of lymph node involvement (P = .045) were associated with improved disease-specific survival and overall survival, respectively. In the Surveillance, Epidemiology, and End Results Database, the median survival time was 23 months. Significantly superior survival was observed in patients with earlier American Joint Committee on Cancer stage (Ⅰ-Ⅱ) (P < .001 for both overall survival and disease-specific survival). Patients who were diagnosed within the recent decade did not achieve a better survival (P = .599 for overall survival and P = .596 for disease-specific survival).ConclusionsPPSS was aggressive with a very poor prognosis. The seventh American Joint Committee on Cancer stage might aid in predicting survival. Pneumonectomy and lymph node involvement might be associated with worse survival, whereas single lobe involvement and postoperative chemotherapy might be associated with improved survival.
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Project description:IntroductionSynovial sarcoma is one of the most common soft tissue sarcomas in children. Guidelines regarding the adequate extent of resection margins and the role of re-resection are lacking. We sought to evaluate the adequate resection margin and the role of re-resection in predicting outcomes in children with synovial sarcomas.MethodsA cohort of 36 patients less than 18 years of age at diagnosis who were treated for localized synovial sarcoma at three tertiary pediatric hospitals between January 2004 and December 2020 were included in this study. Patient and tumor demographics, treatment information, and margin status after surgical resection were collected from the medical record. Clinical, treatment, and surgical characteristics, as well as outcomes including hazard ratios (HRs), event-free survival (EFS), and overall survival (OS) were compared by resection margins group and re-resection status.ResultsPatients in the R1 resection group were significantly more likely to relapse or die compared to patients in the R0 resection group. However, there was no significant difference in EFS (HR 0.52, p = 0.54) or OS (HR 1.56, p = 0.719) in R0 patients with less than 5 mm margins compared to R0 patients with more than 5 mm margins. Patients with R1 on initial or re-resection had significantly worse OS than patients who had R0 resection on initial or re-resection (HR = 10.12, p = 0.005).ConclusionThis study re-affirms that R0 resection is an independent prognostic predictor of better OS/EFS in pediatric synovial sarcoma. Second, our study extends this finding to report negative margins on initial resection or re-resection is associated with better OS/EFS than positive margins on initial resection or re-resection. Lastly, we found that there is no difference in outcomes associated with re-resection or <5 mm margins for R0 patients, indicating that re-resection and <5 mm margins are acceptable if microscopic disease is removed.
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Project description:BackgroundPrimary cardiac synovial sarcoma is a rare condition with limited treatment options for advanced stages. Surgery and chemotherapy are currently the mainstay treatments; however, survival rates remain low.Case presentationA 64-year-old woman presenting with symptoms of chest tightness and shortness of breath was found to have an obstructive right atrial mass, along with pulmonary infarction and metastasis. She was ultimately diagnosed with advanced primary cardiac synovial sarcoma. Following surgery, the patient's symptoms improved, and she underwent chemotherapy and anti-angiogenic therapy, but unfortunately, her survival time was only 8 months.Conclusion This case report aims to enhance clinicians' understanding of the diagnosis and treatment of primary cardiac synovial sarcoma. Enhancing both survival outcomes and quality of life in individuals with primary cardiac synovial sarcoma continues to present a significant challenge.
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Project description:Intrathoracic synovial cell sarcomas are extremely rare tumors with a high rate of recurrence after resection. We report one such case of synovial cell sarcoma arising from the chest wall, which was completely intrathoracic without an external component. The tumor was resected twice and in spite of adjuvant chemo-radiation the tumor recurred. Radical resection of the tumor with reconstruction of the chest wall and diaphragm using titanium mesh and polypropylene mesh respectively was done. A latissimus dorsi flap cover was provided. Titanium mesh is an excellent prosthesis of choice for reconstruction, as it provides stability and better cosmesis of the chest wall. Radical resection can be offered for suitable candidates, with very low morbidity and mortality.Supplementary informationThe online version contains supplementary material available at 10.1007/s12055-022-01378-3.
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