Coexistence of a Rare Giant Right Ventricular Angiosarcoma with Right Coronary Artery Pseudoaneurysm and Unilateral Pleural Effusion: A Case Report

Introduction

Angiosarcoma is a rare malignant tumor from vascular endothelial cells covering lymphatic or blood vessels. Cardiac angiosarcoma is the most prevalent sarcoma in the heart [1] [2]. It has a low incidence rate and poor prognosis. The size and location of the tumor and its degree of invasion are some points on which clinical manifestations strongly depend [3] [4]. Only 20% of Primary Cardiac tumors are malignant, and 80% are benign. Cardiac angiosarcomas are scarce sarcomas of soft tissue and have a poor prognosis [1] [2] [3] [4]. It is a life-threatening condition that has a poor prognosis due to the limited treatment options and its extraordinarily aggressive behavior, even when complete resection of the tumor is performed [5] [6]. The prevalence of primary cardiac angiosarcomas is higher in males in a two-thirds ratio. Diagnosis is usually hindered because this tumor is typically associated with a variety of nonspecific symptoms [1] [3]. Approximately 80% of cardiac angiosarcomas present metastatic spread at diagnosis [6]. Surgery is not usually an effective treatment because of the large mass of cardiac tissue invasion or the presence of metastasis [6] [7]. As a standardized management, complete or partial surgery resection is still the best palliative option. However, chemotherapy and target therapy are bound [1] [7]. Herein, we describe a patient with metastatic cardiac angiosarcoma, which started as a highly soft mass density before the initial diagnosis. However, once the correct diagnosis was made, the mass had already infiltrated in the anterior mediastinum, surrounding the right atrium and forming a pseudoaneurysm in the right coronary artery. This case underscores the importance of comprehensive imaging in early detection and highlights the limited therapeutic options available for cardiac angiosarcoma with extensive vascular involvement. Prompt diagnosis and a multidisciplinary approach are crucial to manage the aggressive progression of these tumors.

Publication History

Received: 06 July 2025

Accepted after revision: 18 August 2025

Article published online:
01 September 2025

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