Successful surgical excision of primary right atrial angiosarcoma

Wobbe Bouma,Tineke P Willems,Massimo A Mariani,Iwan Cc Van Der Horst,Tjark Ebels,Albert Jh Suurmeijer,Chris Ph Lexis

doi:10.1186/1749-8090-6-47

Wobbe Bouma, Tineke P Willems + Show 5 more

Open Access

PDF Available

https://doi.org/10.1186/1749-8090-6-47

Copy DOI

Export

Save

Cite

Abstract
Highlights/Summary
Full-Text PDF
Similar Papers

Abstract

Listen

Primary cardiac angiosarcoma is a rare and aggressive tumor with a high incidence of metastatic spread (up to 89%) at the time of diagnosis, which restricts the indication for surgical resection to a small number of patients. We report the case of a 50-year old Caucasian woman with non-metastatic primary right atrial angiosarcoma, who underwent successful surgical excision of the tumor (with curative intent) and reconstruction of the right atrium with a porcine pericardial patch. However, after a symptom-free survival of five months the patient presented with bone and liver metastases without evidence of local tumor recurrence.

Highlights

Angiosarcomas, rare, are the most common primary malignant neoplasms of the heart [1,2]
In this report we describe the case of a 50-year old Caucasian woman with non-metastatic primary right atrial angiosarcoma, who underwent successful surgical excision of the tumor and reconstruction of the right atrium with a porcine pericardial patch
The tumor cells were positive for the endothelial markers factor VIII-related protein, CD31 and CD34 (Figure 3B), whereas reactivity to podoplanin, smooth muscle actin, desmin, S100 protein, keratins, and EMA was negative. These findings confirmed the diagnosis of primary right atrial angiosarcoma

Summary

Introduction

Angiosarcomas, rare, are the most common primary malignant neoplasms of the heart [1,2]. Symptoms are often nonspecific and absent for a long time, primary cardiac angiosarcomas (PCAs) may present abruptly at which point there is often already metastatic spread [2,3] This restricts the indication for surgical resection to a small number of patients. The tumor cells were positive for the endothelial markers factor VIII-related protein, CD31 and CD34 (Figure 3B), whereas reactivity to podoplanin, smooth muscle actin, desmin, S100 protein, keratins, and EMA was negative These findings confirmed the diagnosis of primary right atrial angiosarcoma. After a symptom-free survival of five months the patient presented with bone and liver metastases without evidence of local tumor recurrence.

Findings

Discussion

Conclusions