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Primary pericardial angiosarcoma: A case report

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Abstract

Primary pericardial angiosarcoma is a rare malignant cardiac neoplasm with early metastasis and poor prognosis. There are currently no guidelines or effective therapeutic strategies. Here we report a case of a 22-year-old man who presented with chest pain, suffocation and transient syncope over the course of 4 months. Further workup showed a large mass in the right pericardium, histopathologic examination revealed angiosarcoma. The patient subsequently received a total of 8 cycles of chemotherapy (paclitaxel and doxorubicin). This patient has an overall survival of 1 year to date. The current examination methods and reported cases revealed that early detection of primary pericardial angiosarcoma with imaging examinations is critical for prognosis.

Introduction

Cardiac angiosarcoma (AS) is the most common primary malignant cardiac tumor in adults.1 However, primary pericardial angiosarcoma is extremely rare. The diagnosis is often delayed due to nonspecific clinical symptoms. Owing to the aggressiveness of angiosarcoma, its prognosis is predominantly poor. The median overall survival (OS) ranging from 6 to 14 months.2 Herein, we report a case of an extensive pericardial angiosarcoma involving the right atrium and review relevant literatures to explore its imaging characteristics.

Section snippets

Case report

A 22-year-old man presented at a nearby hospital with chest pain and stuffiness, syncope. Transthoracic echocardiography (TTE) revealed massive pericardial effusion. The effusion culture for bacteria, acid-fast bacilli and viruses were negative, as well as the examination for malignant cells. Chest radiograph demonstrated the pneumonia of both lungs. His symptoms were relieved after pericardial effusion drained and anti-inflammatory treatment. Three months later, he presented with chest pain

Discussion

The incidence of primary cardiac tumor is low, which is reported as 0.0017%-0.033%.3 Angiosarcoma is the most common primary malignant cardiac tumor, it often occurs in those 30-50 years of age and the male to female ratio is (2-3):1. Right atrium is the most predilection site of cardiac angiosarcoma. The clinical symptoms and signs of primary cardiac angiosarcoma were not specific, so it’s hard to diagnose. Cardiac angiosarcoma often metastasizes to the lungs and bones.

The tumor detection

Conclusion

Primary pericardial angiosarcoma is extremely rare. The diagnosis is often delayed due to nonspecific clinical symptoms. Multimodality imaging can provide additional information for clinicians to timely diagnose and deliver adequate treatment to their patients.

Disclosures

Wencui Li, Lizhu Han and Zhaoxiang Ye declare that they have nothing to disclose.

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