Successful robotic resection of a primary cardiac synovial sarcoma.
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ABSTRACT:
SUBMITTER: Akhmerov A
PROVIDER: S-EPMC8298838 | biostudies-literature |
REPOSITORIES: biostudies-literature
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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 .).
| S-EPMC9434647 | biostudies-literature
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.
| S-EPMC9390277 | biostudies-literature
Project description: Not available
| S-EPMC9735421 | biostudies-literature
Project description: Not available
| S-EPMC7847793 | biostudies-literature
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.
| S-EPMC9390693 | biostudies-literature
Project description:Primary 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. A 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. 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.
| S-EPMC11335867 | biostudies-literature
Project description:Synovial sarcoma is the most common pediatric non-rhabdomyosarcoma soft tissue sarcoma and accounts for about 8-10% of all soft tissue sarcoma in childhood and adolescence. The presence of a chromosomal translocation-associated SS18-SSX-fusion gene is causally linked to development of primary synovial sarcoma. Metastases occur in approximately 50-70% of synovial sarcoma cases with yet unknown mechanisms, which led to about 70-80% mortality rate in five years. To explore the possibilities to investigate metastatic mechanisms of synovial sarcoma, we carried out the first genome-wide search for potential genetic biomarkers and drivers associated with metastasis by comparative mutational profiling of 18 synovial sarcoma samples isolated from four patients carrying the primary tumors and another four patients carrying the metastatic tumors through whole exome sequencing. Selected from the candidates yielded from this effort, we examined the effect of the multiple missense mutations of ADAM17, which were identified solely in metastatic synovial sarcoma. The mutant alleles as well as the wild-type control were expressed in the mammalian cells harboring the SS18-SSX1 fusion gene. The ADAM17-P729H mutation was shown to enhance cell migration, a phenotype associated with metastasis. Therefore, like ADAM17-P729H, other mutations we identified solely in metastatic synovial sarcoma may also have the potential to serve as an entry point for unraveling the metastatic mechanisms of synovial sarcoma.
| S-EPMC6305143 | biostudies-literature
Project description:Primary cardiac sarcomas are very aggressive, being a challenge to cardio-oncology specialty. Surgical planning and innovative techniques have enhanced the possibility of resection. We have described a case of recurrent primary left atrium angiosarcoma, successfully resected with a modified partial autotransplantation technique, planned using a 3-dimensional model created from computed tomography. (Level of Difficulty: Intermediate.).
| S-EPMC8571791 | biostudies-literature
Project description:BackgroundCardiac synovial sarcoma (CSS) is an extremely rare malignant tumour with a poor prognosis. We report the case of a 31-year-old woman who presented with a CSS in the right atrium and was initially misdiagnosed with a tuberculoma. The aim of this article is to focus on the importance of having broad differential diagnoses including rare entities.Case summaryA 31-year-old White woman, with a close contact with a relative having pulmonary tuberculosis, presented to the emergency unit with severe dyspnoea. Chest radiography and echocardiography showed a large pericardial effusion with a mass in the right atrium. Pericardiocentesis removed bloody exudative fluid with adenosine desaminase at 17 UI/L and no malignant cells in the cytological study. Cardiac magnetic resonance revealed a tuberculoma of the right atrium. Intraoperatively, the mass was only biopsied because of the local invasion. Histological study concluded to a CSS. The patient died 3 days later.DiscussionThis case highlights the importance of having broad differential diagnoses including rare entities. Histology was the key investigation for the diagnosis of CSS which has no clinical nor laboratory or imaging pathognomonic signs.
| S-EPMC8186913 | biostudies-literature
Project description: Not available
| S-EPMC5331388 | biostudies-literature