Primary pericardial angiosarcoma: case report and review of treatment options

Yadav, Udit; Mangla, Ankit

doi:10.3332/ecancer.2020.1056

Cited by 10 publications

(11 citation statements)

References 20 publications

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“…The mortality of pulmonary angiosarcoma within the first few months after clinical presentation approaches 100%, most patients die within a few months after the first visit. Due to its rarity and challenges during treatment, clinical studies with large sample sizes to establish standardized guidelines for diagnosis and treatment are difficult to conduct ( 23 , 27 ). Timely diagnosis and early treatment are key to effectively prolonging the survival time of patients.…”

Section: Discussionmentioning

confidence: 99%

A Rare Cause of Pulmonary Nodules Diagnosed as Angiosarcoma Was Misdiagnosed as Vasculitis and Wegener’s Granuloma in an Elderly Man: A Case Report

Wang

Yang

2021

Front. Oncol.

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BackgroundAngiosarcoma is a rare, highly malignant tumor prone to recurrence and metastasis. Angiosarcoma is insidious in the initial stage, and its clinical manifestation lacks specificity. The diagnosis is based on histopathology and immunohistochemistry findings.Case presentationA 73-year-old man was hospitalized following complaints of persistent cough 6 months and hemoptysis for 2 months. Anti-infective treatment was ineffective. A CT-guided percutaneous core needle biopsy of pulmonary lesions revealed organized pneumonia, and the removed skin of purpuric rash area on the left calf revealed vasculitis. Chest CT was used during the patient follow-up. Hormonal therapy combined with immunoglobulins did not lead to improvement, and there was rapid progression of the lung lesions. Subsequently, the patient underwent a surgery, the diseased tissue was separated and removed completely beside the left submandibular gland under local anaesthesia. The immunohistochemical staining indicated CD31 (+) and CD34 (+) confirming a diagnosis of metastatic angiosarcoma. The expression of PD-L1 was 70%, therefore, anlotinib and pembrolizumab treatments were initiated. The patient eventually died.ConclusionAngiosarcoma is a malignant tumor in the clinic that lacks typical and specific signs and symptoms. The diagnosis depends on immunohistochemistry, which requires repeated biopsies of multiple sites in highly suspected cases.

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Section: Discussionmentioning

confidence: 99%

A Rare Cause of Pulmonary Nodules Diagnosed as Angiosarcoma Was Misdiagnosed as Vasculitis and Wegener’s Granuloma in an Elderly Man: A Case Report

Wang

Yang

2021

Front. Oncol.

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“…Achieving R0 margins (no microscopic cancer cells in the margins of the specimen from the primary site) is the goal for all patients and greatly determines prognosis (median survival of 17 months). This drops to six months with R1 margins (microscopic cancer cells present in the margins) [14][15][16][17][18]. However, the proximity of all lesions to vital cardiac structures makes surgical treatment challenging.…”

Section: Discussionmentioning

confidence: 99%

“…Chemotherapy in both adjuvant and neoadjuvant settings has been tried with anthracyclines and taxanes. Neither has been compared in a prospective trial, but retrospective studies have shown similar survival benefits [18][19][20].…”

Section: Discussionmentioning

confidence: 99%

Pericardial Angiosarcoma: A Diagnostic Challenge

Kumaran¹,

Asif²,

Hussain³

et al. 2021

Cureus

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Cardiac angiosarcomas are the most common malignant primary cardiac tumors accounting for 31% of all primary cardiac tumors. However, primary pericardial angiosarcomas are extremely rare and are associated with high mortality.A 41-year-old male with a past medical history of end-stage renal disease (ESRD) on hemodialysis, follicular thyroid carcinoma, hypertension, and asthma presented with recurrent pericardial effusions. Previously, different imaging modalities had shown small hemodynamically stable pericardial effusions with pericardial thickening. His pericardial effusion was attributed to his ESRD until this presentation. However, during the current admission, chest X-ray showed cardiomegaly with lobulated left heart border. Computed tomography (CT) and transthoracic echocardiogram showed an increased posterior complex pericardial effusion when compared to previous imaging. A pericardial window was created and the space was evacuated. Pericardial fluid cytology reported rare atypical cells, which were indeterminate for malignancy. Histopathology from the pericardial biopsy revealed fibrous tissue with cellular proliferation, consistent with an angiosarcoma. A positron emission tomography revealed findings of angiosarcoma (hypermetabolic pericardial soft tissue nodularity and complex pericardial fluid) limited only to the pericardium. Unfortunately, the angiosarcoma was deemed unresectable, and the patient underwent one cycle of chemotherapy with paclitaxel. The patient's hospital course was further complicated by myelosuppression from chemotherapy and disseminated intravascular coagulation. Therefore, no further chemotherapy was pursued. A repeat echocardiogram showed constriction of both ventricles with loculated pericardial effusion. The patient eventually transitioned to comfort care and passed away.Through this case report, we would like to highlight that multiple confounders can be present when considering the etiology of persistent pericardial effusions. We suggest contemplating alternate diagnosis, such as malignancy, and initiate aggressive work-up especially in young individuals with recurrent, unexplained pericarditis.

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“…It had this type of infiltration in another reported case [ 3 ]. It was reported in other locations, including the right atrium [ 6 , 7 ], the left atrium [ 4 ], the pericardium [ 7 ], the aorta [ 8 ], the ventricular outflow tract (RVOT) and main pulmonary artery (MPA) [ 5 ]. Angiosarcomas arising from saphenous vein femoropopliteal bypass grafts have also been reported [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

Resection of primary cardiac angiosarcoma infiltrating the right atrioventricular junction and tricuspid valve: a case report

Bakr

AlKhalaf

Takriti

2021

J Cardiothorac Surg

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Background Primary cardiac tumours are extremely rare. Most of them are benign. Sarcomas account for 95% of the malignant tumours. Prognosis of primary cardiac angiosarcoma remains poor. Complete surgical resection is oftentimes hampered when there is extensive tumour involvement into important cardiac apparatus. We report a case of cardiac angiosarcoma of the right atrium and ventricle, infiltrating the right atrioventricular junction and tricuspid valve. Case presentation Initially, a 22-year-old man presented with dyspnoea. One year later, he had recurrent pericardial effusion. Afterwards, echocardiography revealed a large mass in the right atrium, expanding from the roof of the right atrium to the tricuspid valve. The mass was causing compression on the tricuspid valve, and another mass was seen in the right ventricle. Complete resection of the tumour was impossible. The mass was resected with the biggest possible margins. The right atrium was reconstructed using heterologous pericardium. The patient’s postoperative course was uneventful. Postoperative echocardiography showed a small mass remaining in the right side of the heart. Histopathology and immunohistochemistry confirmed the diagnosis of angiosarcoma. The patient underwent adjuvant chemotherapy and radiotherapy later on. He survived for 1 year and 5 days after the surgery. After a diagnosis of lung and brain metastases, he ended up on mechanical ventilation for 48 h and died. Conclusions Surgical resection combined with postoperative chemotherapy and radiotherapy is feasible even in patients with an advanced stage of cardiac angiosarcoma when it is impossible to perform complete surgical resection.

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Primary pericardial angiosarcoma: case report and review of treatment options

Cited by 10 publications

References 20 publications

A Rare Cause of Pulmonary Nodules Diagnosed as Angiosarcoma Was Misdiagnosed as Vasculitis and Wegener’s Granuloma in an Elderly Man: A Case Report

A Rare Cause of Pulmonary Nodules Diagnosed as Angiosarcoma Was Misdiagnosed as Vasculitis and Wegener’s Granuloma in an Elderly Man: A Case Report

Pericardial Angiosarcoma: A Diagnostic Challenge

Resection of primary cardiac angiosarcoma infiltrating the right atrioventricular junction and tricuspid valve: a case report

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