Why the study?
Primary epithelioid angiosarcoma of the pericardium is a rare soft tissue malignancy with aggressive tumor biology, posing diagnostic and therapeutic challenges.
Primary epithelioid angiosarcoma of the pericardium is a rare, aggressive malignancy with poor prognosis despite immunotherapy and chemotherapy.
Portends poor survival in pericardial epithelioid angiosarcoma despite initial chemotherapy response; leaves open optimal treatment paradigms.
A primary epithelioid angiosarcoma of the pericardium is a rare soft tissue malignancy. This report describes a young adult woman who presented with progressive dyspnea and pericardial effusion. She was found to have pericardial mass on imaging along with extensive myocardial infiltration. The biopsy of the mass revealed epithelioid angiosarcoma, which was CD34 and CD31-immuno-positive. Due to unresectable disease, she was given a trial of immunotherapy followed by conventional chemotherapy. She showed partial response on interim assessment, but progressed soon after the completion of six cycles and succumbed to her rapidly progressive illness within nine months of diagnosis. This report discusses the diagnostic and therapeutic challenges faced while managing this disease of aggressive tumor biology.
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Guleria et al. (2024) studied this question.
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