Primary cardiac hemangioendothelioma: early diagnosis and surgical resection

Abstract

Background: The term hemangioendothelioma encompasses all tumors that derive from the endothelium of blood vessels. It has an uncertain prognosis, and it is always considered as a low-grade malignancy.

Clinical case: Male, 23, who was admitted to the emergency room with chest pain, intermittent paroxysmal nocturnal dyspnea and malaise. He denied having a history of degenerative diseases, and had a weight loss of 6 kg in two months. Chest X-rays suggested pericardial effusion. Patient presented a tendency to hypotension tamponade, which was solved with subxiphoid puncture, and drain 800 mL of liquid from ancient hematological parameters. He presented a tumor in the right atrium of 8 x 4 cm, attached to the anterior wall of the atrium without involucre of interatrial septum. When patient underwent surgery, it was identified a tumor lesion in the anterior wall of right appendage. It was done the resection of the tumor and of 70 % of the right atrial appendage. The latter was replaced with bovine pericardium. Patient showed good outcome; it was discharged after 10 days of follow-up surgery for six months in the outpatient clinic. 

Conclusion: The hemangioendothelioma is a vascular tumor of unpredictable behavior, and whose origin rarely comes from the heart. A timely detection, and a radical surgical resection is, so far, the more acceptable management, given the worldwide little experience for handling this type of tumor. Our experience suggests an aggressive clinical approach and surgical removal within the first hours of the suspected diagnosis for the greatest chance of complete resection and reducing the risk of recurrence.