Abstract:Introduction
Primary cardiac synovial sarcoma is an exceedingly rare soft tissue malignant tumor, involving either the pericardium or chambers, with a striking male predominance, prevalently seen in the fourth decade. These tumours most commonly present as breathlessness on exertion. These patients readily exhibit gastrointestinal and systemic symptoms but sometimes the presentation can be fallacious.
Methods
A 37‐year old male presented with fever, breathlessness on exertion and hematuria who was managed for … Show more
“…The platelet count returned rapidly to normal after tumour excision. 9 In our case, the temporary platelet count improvement with immunoglobulin treatment supports an autoimmune-like disorder. The presence of increased LDH and schistocytes suggests a component of mechanical destruction.…”
Section: Discussionsupporting
confidence: 61%
“…Arrhythmias or pericardial effusions can occur due to local invasion. 8 , 9 A right-sided tumour can embolize, causing a pulmonary embolize, while left-sided tumours can embolize into the systemic circulation, causing stroke or other end-organ damage. Some tumours can cause systemic or constitutional symptoms.…”
Background
Primary cardiac sarcomas are rare, aggressive types of malignancies with poor prognoses and can rarely present with thrombocytopenia. Sarcomas account for 65% of primary malignant cardiac tumours. Clinical symptoms often present with constitutional symptoms such as shortness of breath, weight loss, and fatigue. In addition, the tumour’s location determines treatment options and prognosis. Multimodal imaging facilitates the detection and assessment of cardiovascular tumours. This case study presents a rare primary right heart cardiac sarcoma presenting with thrombocytopenia.
Case summary
An 80-year-old male presented to the emergency department with complaints of worsening dyspnoea, ease of bruising, and chest pain. An extensive investigation into the cause of thrombocytopenia was performed. A transthoracic echocardiogram, computed tomography scan, and cardiac magnetic resonance (CMR) image revealed a large mass affecting the right atrium and right ventricle. Myocardial biopsy showed high-grade angiosarcoma. Due to his advanced age and intraventricular septal involvement of the mass, the multidisciplinary team decided to proceed with palliative chemotherapy.
Discussion
Many cardiac tumours remain asymptomatic, and the diagnosis is made at an advanced stage of the disease. Differential diagnoses of the intramural masses include haemangiomas, lipomas, rhabdomyomas, lymphomas, and sarcomas. Multiple treatment options should be considered to address thrombocytopenia. Tumour diagnosis and identification consist of laboratory tests and multimodal imaging. Complete surgical resection with neoadjuvant and adjuvant purposes is the mainstay of cardiac sarcoma therapy. A multidisciplinary, individualized care approach should be performed.
“…The platelet count returned rapidly to normal after tumour excision. 9 In our case, the temporary platelet count improvement with immunoglobulin treatment supports an autoimmune-like disorder. The presence of increased LDH and schistocytes suggests a component of mechanical destruction.…”
Section: Discussionsupporting
confidence: 61%
“…Arrhythmias or pericardial effusions can occur due to local invasion. 8 , 9 A right-sided tumour can embolize, causing a pulmonary embolize, while left-sided tumours can embolize into the systemic circulation, causing stroke or other end-organ damage. Some tumours can cause systemic or constitutional symptoms.…”
Background
Primary cardiac sarcomas are rare, aggressive types of malignancies with poor prognoses and can rarely present with thrombocytopenia. Sarcomas account for 65% of primary malignant cardiac tumours. Clinical symptoms often present with constitutional symptoms such as shortness of breath, weight loss, and fatigue. In addition, the tumour’s location determines treatment options and prognosis. Multimodal imaging facilitates the detection and assessment of cardiovascular tumours. This case study presents a rare primary right heart cardiac sarcoma presenting with thrombocytopenia.
Case summary
An 80-year-old male presented to the emergency department with complaints of worsening dyspnoea, ease of bruising, and chest pain. An extensive investigation into the cause of thrombocytopenia was performed. A transthoracic echocardiogram, computed tomography scan, and cardiac magnetic resonance (CMR) image revealed a large mass affecting the right atrium and right ventricle. Myocardial biopsy showed high-grade angiosarcoma. Due to his advanced age and intraventricular septal involvement of the mass, the multidisciplinary team decided to proceed with palliative chemotherapy.
Discussion
Many cardiac tumours remain asymptomatic, and the diagnosis is made at an advanced stage of the disease. Differential diagnoses of the intramural masses include haemangiomas, lipomas, rhabdomyomas, lymphomas, and sarcomas. Multiple treatment options should be considered to address thrombocytopenia. Tumour diagnosis and identification consist of laboratory tests and multimodal imaging. Complete surgical resection with neoadjuvant and adjuvant purposes is the mainstay of cardiac sarcoma therapy. A multidisciplinary, individualized care approach should be performed.
“…Cardiac SS is an extremely rare and aggressive malignancy accounting for 3-5% of heart tumors with a significant male predominance and right heart location [2,50,51,52]. The most common cardiac location is pericardium with atrium being the second one [52].…”
Section: Synovial Sarcomamentioning
confidence: 99%
“…Cases of atrial synovial sarcoma[2,3,6,50,51,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71] …”
Primary cardiac sarcomas are extremely uncommon. We report two patients with primary cardiac atrial sarcomas: a case report of a 34-year old woman with intimal sarcoma of the left atrium and a case report of a 30-year old man with synovial sarcoma of the right atrium. Clinicopathological and differential diagnosis with a discussion regarding the role of molecular studies is presented.
“…Patients with PCL typically present with dyspnoea and constitutional symptoms such as weight loss and anorexia [2]. Trombocytopenia has rarely been described in association with PCL [3] and other primary cardiac tumours [4][5][6][7][8] and can add substantial morbidity to the diagnosis if present. We report to our knowledge, the second case of primary cardiac lymphoma presenting with marked thrombocytopenia which subsequently resolved with chemotherapy.…”
Primary cardiac lymphoma (PCL) is a rare, potentially fatal subtype of non-Hodgkin’s lymphoma. Thrombocytopenia has also infrequently been reported in association with other primary cardiac tumours and can add substantial morbidity to an already life-threatening diagnosis if present. We report a rare case of a 70-year-old man who presented with thrombocytopenia (91 × 109/L) and progressive right heart failure. Transthoracic echocardiogram revealed a large 8 × 4 cm right atrial mass with severe tricuspid obstruction, confirmed as PCL on subsequent endomyocardial biopsy and immunohistochemistry. He deteriorated into cardiogenic shock precipitated by atrial fibrillation, with worsening thrombocytopenia (18 × 109/L) in the setting of ischaemic hepatitis. The patient stabilised with initiation of high dose steroids prior to tissue diagnosis and platelet counts normalised following chemotherapy. This case demonstrates the importance of considering PCL as a diagnosis and preemptive initiation of high dose steroids to improve outcomes in PCL associated with cardiogenic shock. This case also elucidates a potential pathophysiological association between PCL and thrombocytopenia.
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