A rare cardiac neoplasm: case report of cardiac epithelioid angiosarcoma

Matzke, Lise; Knowling, Margaret A.; Grant, Dennis R.; Cupples, James B.; Leipsic, Jonathon; Ignaszewski, Andrew; Allard, Michael F.

doi:10.1016/j.carpath.2010.09.006

Cited by 9 publications

(8 citation statements)

References 26 publications

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“…Prevalence of primary angiosarcomas of the right atrium is extremely rare [1]. These malignant cardiac tumors are highly aggressive with generally poor prognosis.…”

Section: Discussionmentioning

confidence: 99%

“…Angiosarcomas are malignant tumors that originate from the vascular endothelium. Incidence of angiosarcoma is extremely low [1], but angiosarcomas belong to the most frequently occurring and rapidly spreading primary cardiac tumor [2] Right heart is the usual location of the involvement of the disease, mostly in the right atrium, which is opposed to benign tumors which are usually left sided tumors. The majority of patients exhibit evidence of pericardial disease and/or cardiac failure [3].…”

Section: Introductionmentioning

confidence: 99%

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Fast growing angiosarcoma of the right atrium after radiofrequency catheter ablation: a missed diagnosis or misdiagnosis case report

Wang

Sun

et al. 2020

BMC Cancer

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Background: Primary angiosarcomas of the right atrium are extremely rare, often resulted in missed diagnosis or misdiagnosis with routine examination tools. These malignant cardiac tumors are highly aggressive with generally poor prognosis. Surgical excision is the mainstay of treatment as it is essentially not responsive to current regimens of chemoradiotherapy. Case presentation: Herein, we describe a patient who initially presented with paroxysmal atrial fibrillation and was subsequently treated with radiofrequency catheter ablation (RFCA). Prior to RFCA, an initial transesophageal echocardiography revealed a local thickening of the intratrial septum. Three months later, she was hospitalized with progressive dyspnea and massive pericardial effusion. A large immobile, non-pedunculated mass, occupying almost half of the right atrium was detected by transthoracic and transesophageal echocardiogram. Multimodality cardiac imaging was useful in further characterizing this mass, which was ultimately diagnosed as an angiosarcoma based upon biopsy results. The growth rate was extremely rapid following RFCA, and patient underwent surgical excision. After discharge, the angiosarcoma recurred and patient survived for 7 months from the first episode of tamponade. Conclusions: Primary cardiac angiosarcoma of the right atrium can easily be mistaken for structural anomalies in its early stages, losing the opportunity for initiating earlier treatments to improve potential patient outcomes. The correct diagnosis of this rare case relied on the comprehensive utilization of multimodal imaging techniques including biopsy.

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“…Prevalence of primary angiosarcomas of the right atrium is extremely rare [1]. These malignant cardiac tumors are highly aggressive with generally poor prognosis.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Fast growing angiosarcoma of the right atrium after radiofrequency catheter ablation: a missed diagnosis or misdiagnosis case report

Wang

Sun

et al. 2020

BMC Cancer

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“…There are reports of cardiac angiosarcoma in literature, most of which involved the right atrium with metastasis to the lung. The most prevalent symptoms were dyspnea, chest pain, tachycardia, pleural and pericardial effusion (7,(14)(15)(16)(17)(18)(19). There were also cases presented with left atrium mass (20,21), ascites (16), SVC involvement (8, 19,22,23), valvular complications (18, 20), tamponade and shock (24,25), involvement of coronary vessels (26, 27), or heart rhythm abnormalities (20).…”

Section: Case Presentationmentioning

confidence: 99%

Primary Cardiac Angiosarcoma With Pulmonary Metastasis in a Young Female: A Case Report and Review of Literature

Kordjazy

Taheri

Parkhideh

et al. 2022

Preprint

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Background: Sarcomas are the most prevalent type of malignant primary cardiac tumor. Clinical presentation differs according to the size and location of the tumor and involvement of other structures. Case presentation: In this article, we present a 38 years old lady as a case of primary cardiac angiosarcoma with a huge challenge in diagnosis and management of the disease. On the 4th of June 2020, she emergently presented to the hospital with the chief complaint of cough and tachypnea, diarrhea, and malaise, and a history of blunt chest trauma three weeks ago. Based on the presentation and transthoracic echocardiography which was compatible with cardiac tamponade, emergent pericardiocentesis was performed. The first imaging showed bilateral pleural effusion, pericardial effusion, and no significant parenchymal lung involvement, which was not compatible with rheumatologic diseases or COVID-19. Based on cardiac CT angiography and evidence of dye entrance to the peri-right atrial area, right atrial free wall rupture was suspected as the cause of the massive pericardial effusion. Following multidisciplinary consensus, she underwent cardiac surgery; in addition to the 3*3 cm right atrium free wall rupture repair, concomitant sampling from the lung nodules, lymph nodes, pericardium, and also the surrounding tissue of the right atrium ruptured defect was performed. Based on immunohistochemistry, the diagnosis was compatible with cardiac angiosarcoma with metastasis to the lung. Due to the advanced stage of the tumor, oncologists advised against chemotherapy or radiotherapy and she went through palliative care. Finally, after a 53-day ICU stay and due to right-sided heart failure, the patient unfortunately died. Conclusion: COVID-19 pandemic has brought diagnostic challenges regarding differentiating SARS-CoV-2 infection from other diagnostic entities. On the other hand, due to the rarity and fatality of primary cardiac angiosarcomas, early diagnosis and possible management seem crucial for prolonged survival. Involving cardiac angiosarcoma in the initial differential diagnosis could warrant timely diagnosis and assessment of various therapies for cure or palliative care.

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“…Primary cardiac epithelioid angiosarcoma is extremely rare; to our knowledge, there are only another seven documented reports of this type of tumor [4, 5]. Herein, we present a case of a primary cardiac epithelioid angiosarcoma and a review of the literature.…”

Section: Introductionmentioning

confidence: 95%

Primary Cardiac Epithelioid Angiosarcoma in a Latin American Patient: Case Report and Literature Review

Morgenstern-Kaplan

Aboitiz-Rivera

Blachman‐Braun

et al. 2019

Case Reports in Oncological Medicine

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Cardiac angiosarcoma is a rare and clinically challenging pathology. It is a high-grade primary malignant tumor of the heart tissue that has many variants, of which the epithelioid variant is rarely present in the heart or the great vessels. As with many other cardiac tumors, it is mainly a diagnosis of exclusion and the initial diagnostic test is an echocardiogram followed by a biopsy with immunohistochemistry analysis to ascertain the type of tumor. The differential diagnosis of cardiac tumors is challenging due to the overlapping clinical manifestations with different cardiac tumors and systemic diseases. Cardiac angiosarcomas are often aggressive with a poor prognosis even with treatment. Herein, we present a case of the epithelioid variant of a cardiac angiosarcoma in addition to a thorough review of the recent literature on the clinical manifestation, diagnosis, and treatment of this type of tumors.

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A rare cardiac neoplasm: case report of cardiac epithelioid angiosarcoma

Cited by 9 publications

References 26 publications

Fast growing angiosarcoma of the right atrium after radiofrequency catheter ablation: a missed diagnosis or misdiagnosis case report

Fast growing angiosarcoma of the right atrium after radiofrequency catheter ablation: a missed diagnosis or misdiagnosis case report

Primary Cardiac Angiosarcoma With Pulmonary Metastasis in a Young Female: A Case Report and Review of Literature

Primary Cardiac Epithelioid Angiosarcoma in a Latin American Patient: Case Report and Literature Review

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