Jpn J Thorac Cardiovasc Surg (2006) 54:528–531
DOI 10.1007/s11748-006-0063-9

CASE REPORT

Primary cardiac angiosarcoma: presenting with cardiac tamponade
followed by cerebral hemorrhage with brain metastases
Eriko Ikeya, MD · Junichi Taguchi, MD
Masaomi Yamaguchi, MD · Makoto Shibuya, MD
Kazuo Kanabuchi, MD

Received: 12 July 2005 / Accepted: 19 July 2006
© The Japanese Association for Thoracic Surgery 2006

Abstract A 49-year-old man presented with palpitation
and shortness of breath. He was seen to have a massive
pleural and pericardial effusion on radiography and
echocardiography. Computed tomography (CT) scanning showed that cardiac tumors arose from the
right atrium with epicardial and endocardial extension.
Pathology examination of samples at pericardiotomy
revealed them to be angiosarcoma. Two days after the
surgery, he developed left hemiparesis. CT scans showed
a large cerebral hemorrhage on the right temporal lobe
with midline shift by brain metastases. He died 37 days
after the surgery. At autopsy, he had metastases in the
brain, multiple bones, and soft tissues but no lung or leftside heart involvement. Primary cardiac angiosarcoma is
rare, and mostly arises from the right side of the heart.
Common metastatic sites are the lungs and liver. There
are only a few reports of brain metastases. In conclusion,
this is a rare report of cardiac angiosarcoma presenting
with pericardial tamponade. There were rapid brain and

E. Ikeya (*) · M. Yamaguchi
Department of Cardiovascular Surgery, Tokai University
Hospital, Boseidai, Isehara, Kanagawa 259-1193, Japan
Tel. +463-93-1121; Fax +463-95-7567
e-mail: esatou-cvs@umin.ac.jp
J. Taguchi
Department of Cardiology, Tokai University Hachioji Hospital,
Kanagawa, Japan
M. Shibuya
Department of Pathology, Tokai University Hachioji Hospital,
Kanagawa, Japan
K. Kanabuchi
Department of Cardiovascular Surgery, Tokai University
Hachioji Hospital, Kanagawa, Japan

multiple bone metastases but no lung or left-side heart
lesions.
Key words Angiosarcoma · Cardiac tamponade · Brain
metastases

Introduction
Primary cardiac sarcomas are rare and constitute less
than 25% of primary cardiac tumors. Metastases are
common and are often widespread. Most patients die
within a few months after symptom onset. We report a
patient with an angiosarcoma that originated in the right
atrium who presented with cardiac tamponade followed
by cerebral hemorrhage with rapid brain metastases.

Case
A 49-year-old man presented with a 2-month history of
palpitation and shortness of breath. He had an episode
of hemorrhage of hypodermal bleeding in his left chest.
He was a regular worker and had no special medical
history. His laboratory tests revealed a slight tendency
of prothrombin time prolongation (14.4; normal 10.7–
13.3).
One month later, he was seen to have a massive
pleural effusion and cardiac tamponade on chest radiography and echocardiography. Chest computed tomography (CT) scans showed cardiac tumors that arose
from the right atrium with epicardial and endocardial
extension (Fig. 1a). We performed exploratory pericardiotomy and removed 400 ml of sanguineous fluid.
Multiple tumors were found over the right atrium and

Jpn J Thorac Cardiovasc Surg (2006) 54:528–531

occupying the pericardial space. The tumors were dark
red and easily hemorrhaged. We chose palliative therapy
and relieved the cardiac tamponade. Two days after
surgery, he suddenly developed left hemiparesis. The
head CT scan showed a large cerebral hemorrhage on his
right temporal lobe with midline shift presumably
caused by a metastatic brain lesion (Fig. 2b). One month

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after surgery, he was found to have multiple bone and
soft tissues metastases by CT. He died 37 days after
surgery.
At autopsy, cardiac tumors were found to have invaded the right atrium, especially the lateral wall, but
not the left atrium or interatrial septum. Disseminated
nodules were present in the right ventricle wall and pericardium (Fig. 3a). Multiple metastases were found in the
right temporal lobe of the brain, subcutaneous tissue of
the left shoulder area with left subclavian artery invasion, the right psoas muscles, and multiple bones (left
clavicle, several lumbar vertebrae) but not the lungs, the
cardiac left atrium, or the cardiac left ventricle.
Microscopicably, the right atrium tumor was composed
of a proliferation of atypical spindle cells forming thinwalled blood vessels filled with numerous erythrocytes
(Fig. 2b). This tumor was also stained positively with the
antibodies for factor VIII-related antigen and CD31 as
the endothelial markers, which was compatible with
angiosarcoma.

Discussion
Fig. 1 Chest Computed tomography (CT) scan showed that the
cardiac tumor (arrow) arose from the right atrium and exhibited
epicardial and endocardial extension

(a)

Primary cardiac angiosarcoma is a rare tumor that
usually originates in the right side of the heart. In adults,

(b)

Fig. 2 At admission, there was no significant lesion apparent on his head CT scan (a). Only 8 days later his head CT scan showed a large
cerebral hemorrhage on the right temporal lobe with a midline shift caused by brain metastasis (b)

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a

Jpn J Thorac Cardiovasc Surg (2006) 54:528–531

b

Fig. 3 a At autopsy, cardiac tumors were seen to have originated
from the right atrial wall and invaded the right ventricle wall and
pericardium. PA, pulmonary artery; Ao, aorta; LA, left atrium b
Angiosarcoma is composed of a proliferation of atypical spindle

cells forming thin-walled blood vessels filled with numerous erythrocytes and stained positively with the antibodies for factor VIIIrelated antigen (arrows)

approximately 25% of primary cardiac tumors are malignant, and one-third of these malignant tumors are
angiosarcomas.2 A review of cardiac angiosarcomas by
Janigan et al. showed that right and left atrium involvement was 93% and 7%, respectively. Central nervous
system metastases were reported in three of nine cases in
their review.5
During our search of articles and reports about primary cardiac angiosarcomas, we found only nine cases
of brain metastasis since the report of Janigan et al.
There were five males and four females (15–58 years old).
Six of the lesions originated in the right atrium, two in
the left atrium, and one in the pericardium. There were
seven angiosarcomas, one fibrosarcoma, and one sarcoma. Five patients had brain metastases after cardiac
surgery with cardiopulmonary bypass, which could have
caused systemic dissemination of tumor cells. Among
the other four patients, three had lung metastases, and
one originated in the left atrium. Therefore, all nine cases
had sufficient cause for systemic metastasis of the tumor
cells.
In our case, on admission to the hospital, the head CT
scan showed no metastases (Fig. 2a). However, cerebral
hemorrhage (probably at a brain micrometastatic site)
occurred only 8 days later without lung or left-side heart
metastatic lesions.
The mechanism of the brain metastasis in our patient
is still unknown. Even after vigorous pathology examination, there was no evidence of direct invasion of the
tumor into the left ventricular wall or the aortic wall.
However, these tumors existed in the pericardial space.

There is still a possibility that the tumor cells invaded
the coronary circulation from the epicardial space.
Furthermore, generalized metastasis might occur via
micrometastases reaching cardiac perforated arterioles
from the coronary circulation to the left ventricular
chamber. Needless to say, this speculation is but one of
the possible mechanisms that can produce of generalized
metastases.
In conclusion, this is a rare case of angiosarcoma that
originated in the right atrium. It presented with massive
pleural effusion, pericardial tamponade, and rapid brain
and multiple bone metastases but with no lung or leftside heart lesions.

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