JIACM 2012; 13(3): 247-51

CASE REPORT

Intra-cardiac angiosarcoma
Madhuchanda Kar*, Rakesh Roy**, Sayan Das***, Alolika Dattagupta****

Abstract
Intra-cardiac angiosarcoma is a challenging disease – difficult to diagnose early and an aggressive tumour to treat. Trans-
oesophageal echocardiography (TEE) and cardiac computed tomography are the two most sensitive diagnostic tools. Though
there are various multimodality treatment options available, the results are not impressive. We present a case of intra-cardiac
angiosarcoma in a 62-year-old gentleman.
Key words: Intra-cardiac, trans-oesophageal echocardiography, angiosarcoma.

Introduction l Enlarged nodes in perivascular region, pre- and sub-

Malignant tumours of the heart and pericardium are rare
with an autopsy prevalence of 0.001 - 0.28%. The most
common primary malignant tumour of the heart and
pericardium is sarcoma. The diagnosis is challenging due
to nonspecific symptoms and an unpredictable
presentation. The prognosis is typically poor. Treatment is
often multidisciplinary, such that the greatest challenge
is how to ensure optimal integration of surgery,
chemotherapy, and radiotherapy. Complete resection is
the ultimate goal, although rarely feasible due to anatomic
reasons and loco-regional spread. Pre-operative
chemotherapy or chemo-radiation may have a role in the
management of unresectable tumours.
Case history
A 62-year-old male residing at Malda district of West
Bengal was suffering from shortness of breath and
heaviness of chest for the last 1 month. He was
normotensive, non-diabetic, with no significant family
history or past history.
carinal region,
l Moderate pericardial effusion,
l Dilated pulmonary trunk,
l Dilated right atrium (RA) with heterogeneous mass
within measuring 7.8 cms X 4 cms occupying most of
the right atrium (Fig. 1) with a few areas of necrosis
within the mass and stasis of intravascular contrast in
the superior vena cava,
l Patchy infiltrate at right apex and plate atelectasis at
left lower lobe.
Trans-oesophageal echocardiography (Fig. 2)
l A large lobulated sessile mass with areas of
calcification and necrosis noted at right atrium
occupying a considerable volume, the mass was
adherent to the postero-lateral free wall of RA and
superior aspect of interatrial septum,
l No flow obstruction; with left ventricular ejection
fraction of 65%,

Haematology and biochemistry reports: Routine
haemogram was within normal range. Liver function test
showed altered albumin: globulin ratio (4.4 gm/dl with
albumin 3.1 gm/dl - A: G ratio 0.74). Total protein was 7.5
gm/dl with negative M spike and increased α1, α2
globulin. CRP was 68.7 mg/dl (< 6 mg/dl).
Endoscopy
Upper GI endoscopy showed normal gastric mucosa with
nodularity at D1 and D2 suggestive of nodular duodenitis.
CT scan of thorax and upper abdomen revealed
l Lytic lesions at D3, D8, D11 vertebrae,
l Large pericardial effusion with no tamponade.
FNAC – proved to be inconclusive for malignancy. He was
advised an open biopsy of the mass under general
anaesthesia. Intra-operative findings showed
haemorrhagic pericardial effusion with a nodular reddish-
brown coloured tumour involving almost whole of the
RA with partial obstruction of SVC and complete
obstruction of inferior vena cava. RA incision was made
and biopsy was taken and chambers were closed in steps
(Fig. 3). Histopathology revealed variable areas of
vasoformative lesions with large pleomorphic cells with
significant mitotic activity forming vascular channels
suggestive of angiosarcoma.

* Consultant Medical Oncologist, Apollo Gleneagles Cancer Hospital, Kolkata - 700 001,
** Consultant Medical Oncologist, *** 3rd Year Resident, **** 2nd Year Resident, Department of Radio-diagnosis,
Cancer Centre Welfare home and Research Institute (CCWHRI), Kolkata-700 063, West Bengal.
Treatment: We planned chemotherapy for him.
Unfortunately he did not turn-up for chemotherapy, nor
for surgery or radiation, and was lost to follow-up.
Discussion
Twenty-five per cent of primary cardiac tumours in adults
are malignant. Sarcomas represent the commonest
histology, accounting for 20% of all cardiac neoplasms.
Still, primary cardiac sarcoma is a rare clinical entity, with
an incidence of 0.0001% in collected autopsy series1. The
low incidence of primary cardiac sarcomas reflects the
overall low incidence of sarcomas in the general
population and the small percentage of body weight of
heart (0.5%) compared with muscle (40%). Angiosarcomas
are the commonest cardiac sarcomas and make up 33%
of cases. Nearly 80% of cardiac angiosarcomas arise as
mural masses in the right atrium.Typically, they completely
replace the atrial wall and fill the entire cardiac chamber.
They may invade adjacent structures (e.g., vena cava,
tricuspid valve).
These tumours are both symptomatic and rapidly fatal.
Extensive pericardial spread and encasement of the heart
often occur. Pericardial angiosarcoma (without myocardial
involvement) occurs rarely.
The majority of cardiac sarcomas occur between the third
and fifth decades of life with a male preponderance (M: F
ratio 2:1). Primary cardiac sarcoma seldom causes
symptoms until late in the course. Most common
symptoms include dyspnoea, chest pain, palpitations,
fever, and myalgia.The clinical presentation is often found
to mimic the more common cardiopulmonary diseases,
usually valvular heart diseases, although the most
frequent presentation is that of right-sided congestive
heart failure.
l Left-sided embolisation may lead to cerebrovascular
accident, peripheral organ infarction, seizures, and
distant metastases.
l Upper extremity and facial congestion (suggestive of
superior vena cava syndrome) and dysphonia may
occur.
Case reports in the literature describe a variety of clinical
manifestations which include arrhythmia, vena caval
obstruction, pericardial effusion with or without features
of tamponade and conduction disturbances. In contrast
with benign tumours, usually located in the left atrium,
malignant tumours are found almost exclusively in the
right heart, particularly in the right atrium.
Most of the reported series of cardiac sarcomas describe
patients with primary cardiac sarcomas and response to
treatment and survival is uncertain. Complete resection
of cardiac sarcoma is difficult, in view of the location and
extent of involvement. Often tumours are so large at the
time of the operation that complete resection cannot be
done. Moreover, up to 80% of patients present with distant
metastases at diagnosis. In general, recommendations for
the treatment of non-metastatic cardiac sarcoma include
exploration for local control of the primary tumour, to
relieve obstructive symptoms and to prolong disease-free
survival. Cardiac sarcomas generally have a poor
prognosis with a median survival of only 6 months.
Applying the general principles of treatment of soft-tissue
sarcomas occurring anywhere else in the body, the most
critical element is complete surgical resection; however,
the location itself is more difficult for obtaining an
adequate margin of resection. The emphasis is on early
detection and diagnosis. Patients with complete resection

Cardiac sarcoma has no pathognomonic physical features

that may be discovered on examination. However, the
following signs may accompany cardiac sarcoma:
m Dyspnoea
m Haemoptysis
m Diminished cardiac sounds
m Friction rub
m Rales
m Refractory arrhythmias
m Heart block
m Heart failure
l An audible plop due to tumour prolapse through the Fig. 1: Computed tomography demonstrating the intra-cardiac mass
(arrow).
AV valve may be appreciated during auscultation.

248 Journal, Indian Academy of Clinical Medicine l Vol. 13, No. 3 l July-September, 2012
Fig. 2: Series of echo-Doppler images demonstrating the mass in the right atrium.

Journal, Indian Academy of Clinical Medicine l Vol. 13, No. 3 l July-September, 2012 249
have a survival of 24 months compared with 10 months
in those with incomplete resection. Orthotopic heart
transplantation could have been performed in selected
patients. However, most are not transplanted because of
the high-risk of tumour recurrence or metastasis and the
possible enhancement of tumour growth by
immunosuppressive drugs.
The use of radiotherapy is also restricted in many ways.
The typical dose of radiation for sarcomas at most sites is
6,000 Cgy to 6,500 Cgy following complete resection of
the sarcoma. In unresectable lesions, the dose of radiation
is often increased to 7,000 Cgy. Such high doses are not
well tolerated by the heart. At a dose of 4,000 Cgy, the
incidence of pericarditis is approximately 40%.
Hyperfractionated radiotherapy (7,050 Cgy) along with a
radiosensitiser (5-iododeoxyuridine) has been shown to
eradicate the tumour in a few cases for locoregional
control, after surgical resection in nonmetastatic tumours.
The role of adjuvant chemotherapy after surgically
resected cardiac sarcoma remains controversial. There is
some evidence to support adjuvant chemotherapy to
relieve symptoms and prolong survival as part of the
combined modality approach. Some other studies, which
Fig. 3: Post-operative scar.
250 Journal, Indian Academy of Clinical Medicine
compared various chemotherapeutic regimes
(cyclophosphamide, vincristine, dacarbazine, ifosfamide,
methotrexate, vincristine, doxorubicin), concluded that
postoperative chemotherapy failed to modify the natural
course of patients with resected cardiac sarcomas. There
has been an isolated report of a cardiac angiosarcoma
that responded to multidisciplinary treatment with
recombinant interleukin-2, post-operative chemotherapy,
and radiation in spite of incomplete resection.
Conclusions
Cardiac angiosarcoma is a rare diagnosis and one that is
often difficult to make because of the nonspecific
presenting signs and symptoms. Initial diagnosis is usually
suggested at echocardiography, but identification of
mediastinal invasion and extracardiac metastases is best
achieved with CT and MR imaging. The overall prognosis
remains poor despite advances in surgical techniques.
There is, however, an encouraging trend towards more
accurate and earlier diagnosis, in large part because of
improved imaging techniques.
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