Resection of a synovial cell sarcoma by cardiac autotransplantation: A case report.
Ontology highlight
ABSTRACT:
SUBMITTER: Mullis DM
PROVIDER: S-EPMC9735421 | biostudies-literature | 2022 Dec
REPOSITORIES: biostudies-literature
Publications
Resection of a synovial cell sarcoma by cardiac autotransplantation: A case report.
Mullis Danielle M DM Zhu Yuanjia Y Guenthart Brandon A BA Bonham Spencer A SA Trope Winston L WL Berry Gerald J GJ Woo Y Joseph YJ MacArthur John W JW
JTCVS techniques 20220920
Similar Datasets
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-EPMC8298838 | biostudies-literature
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: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.
| S-EPMC11861953 | biostudies-literature
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.
| S-EPMC11335867 | biostudies-literature
Project description:BackgroundSurgery is the fundamental method for the treatment of primary cardiac tumours. However, due to the inaccessibility of anatomy and the proximity of important structures, it is very difficult to completely resect tumours of the left atrium or left ventricle without damaging the normal tissues. Cardiac autotransplantation for the resection of cardiac tumours is carried out by taking out the heart from the body, resecting cardiac tumours, and then transplanting the heart back into the body.Case summaryThis article presents a successful case of cardiac autotransplantation for the complete resection of primary cardiac tumour in a 2-month-old infant and shares the noteworthy experience.DiscussionTumours located in the left atrium and left ventricle are difficult to be exposed because of their deep posterior location and proximity to important anatomical structures such as mitral valve and chordae tendineae. How to resect the tumours completely without damaging the normal tissues is a great challenge. This case proves that cardiac autotransplantation is a good solution for tumours that are difficult to be resected completely by orthotopic cardiac transplantation.
| S-EPMC7042140 | biostudies-literature
Project description:IntroductionSynovial sarcoma is a rare, malignant neoplasm accounting for approximately 5-10% of all soft tissue sarcomas. Synovial sarcomas generally occur in the extremities of adolescent or young adult (AYA)-aged patients; however, they have been reported to affect virtually every organ system. The mediastinum is an exceptionally rare location for synovial sarcoma and malignant pericardial effusions occurring secondary to synovial sarcoma can occur.Case presentationThis case describes a 29-year-old male who presented with 3 weeks of dyspnea and leg swelling. An echocardiogram demonstrated a large pericardial effusion with early tamponade physiology. A subsequent pericardiocentesis returned a lymphocytic predominance with negative flow cytometry and cytology. Cross-sectional imaging with computed tomography (CT) scans of the chest, abdomen, and pelvis was negative for lymphadenopathy or detectable mass. He was discharged where repeat echocardiogram demonstrated recurrence of the effusion, but his symptoms had improved. Ultimately, he was readmitted with refractory dyspnea and edema where cardiac magnetic resonance imaging demonstrated a large mediastinal mass invading the pericardium. The biopsy demonstrated a high-grade spindle cell malignancy positive for a rearrangement of the SS18 gene, a finding diagnostic of synovial sarcoma. He passed away after one round of doxorubicin due to septic and obstructive shock (tamponade).ConclusionMediastinal synovial sarcomas are rare, aggressive, and highly morbid neoplasms. In some cases, they may present insidiously due to their sole presenting clinical manifestation being pericardial effusion. Particular care should be taken to rule out malignancy with MRI or PET-guided imaging in the AYA-aged population when idiopathic, recurrent pericardial effusion is found.
| S-EPMC11731899 | biostudies-literature
Project description:Canine joint sarcomas, designated synovial sarcomas, are uncommon malignant mesenchymal neoplasms that occur in the large joints of the extremities of middle-aged, large-breed dogs. We report the diagnosis of an intrathoracic sarcoma with ultrastructural characteristics reminiscent of human synovial sarcoma in a dog.A 7-year-old female spayed Tibetan terrier crossbred dog was presented for acute severe labored breathing and diagnosed with an intrathoracic neoplastic mass. The neoplasm resulted in the accumulation of substantial amounts of viscous pleural fluid that led to dyspnea. The neoplastic mass consisted of interweaving bundles of large pleomorphic mesenchymal cells, supported by an alcian blue positive myxomatous matrix. The neoplastic cells were immunohistochemically negative for cytokeratin and CD18. Transmission electron microscopy indicated that the neoplastic cells had desmosome junctions, short microvilli-like structures and ample amounts of rough endoplasmic reticulum resembling type B-like synoviocytes and synovial sarcoma as reported in people. Despite complete surgical excision of the neoplastic mass, clinical signs recurred after a month and led to the euthanasia of the dog.Currently, there are no immunohistochemical markers specific for synovial sarcoma. Canine neoplasms with transmission electron microscopy characteristics resembling type B-like synoviocytes should be considered similar to the human sarcomas that carry the specific translocations between chromosomes X and 18.
| S-EPMC5559855 | biostudies-other
Project description:Introduction Synovial sarcoma is a malignant tumor of mesenchymal pluripotent cells. Objectives We present a case of synovial sarcoma in the posterolateral wall of the oropharynx. Resumed report The patient, a 23-year-old woman, was admitted with a history of dysphagia and difficulty in breathing for 8 months, resulting in progressive deterioration and onset of snoring, muffled voice, and local pain. An oropharyngeal tumor in the left posterolateral wall touched the base of the ipsilateral tongue. The patient underwent endoscopic pharyngectomy to remove the lesion. Pathologic examination revealed synovial sarcoma with positive margins, and Mohs technique was proposed for margin control. The margins were disease-free, without the need for total laryngectomy. The pharynx was reconstructed with a microvascular forearm flap. The patient developed postoperative stability. Conclusion Despite its name, synovial sarcoma is rarely sourced directly from synovial membranes. It is most commonly found in the vicinity of large joints. The location at the head and neck, a location poor in synovial tissue, is unusual. Synovial sarcoma in the head and neck has an aggressive nature and poor prognosis. Resection with negative margins remains the foundation of therapy, which is not so easily achieved in the head and neck. It is important for the otorhinolaryngologist and head and neck surgeon to be familiar with this aggressive tumor, which carries high mortality and morbidity. The appropriate diagnosis and treatment can improve prognosis and patient survival.
| S-EPMC4296938 | biostudies-other