Primary Osteosarcoma of the Right Heart Ventricle and Atrium; a ...

CASE REPORT
Primary Osteosarcoma of the Right Heart Ventricle
and Atrium; a Case Report
Terje Forslund1, John Melin1, Anders Seppä2
1Department of Medicine 2Department of Pathology, Central Finland Health Care District Hospital,
Jyväskylä, Finland.
Abstract: Most primary malignancies of the heart, among them also osteosarcoma are found in the left and very uncommonly
in the right ventricle. We report a 75-year-old patient with a primary osteosarcoma sited in the right ventricle occluding the
pulmonary outfl ow. The diagnosis was made when the patient was alive, using echocardiography and computerized scan
tomography examinations. Like in previous reports on such malignancies, it was far too late for surgical or other therapeutic
interventions, and the histological diagnosis was made post mortem.
Keywords: primary heart osteosarcoma, ultrasound, computerized tomography
Introduction
Primary benign heart tumours are predominantly found at the left side of the heart (Lowry and McKee,
1972; Silverman 1980) and represent about 75% all heart tumours, and they are mostly atrial myxomas
(Silverman, 1980; Van-der-Salm, 2000). Primary malignant heart tumours, mostly sarcomas, are less
common and constitute about 20 to 25% of all heart tumours (Lowry and McKee, 1972; Silverman,
1980; Vander Salm, 2000). Angiosarcoma seems to be the most common sarcoma (Janigan et al. 1986;
Burke et al. 1992), but primary lymphoma (Somers and Lothe, 1960), plasmacytoma (Torstveit et al.
1977), and malignant mesenchymoma (Bloor, 1978) have been reported. While metastatic malignant
tumours, including metastatic osteosarcomas, are predominantly sited at the right atrium and ventricle,
primary osteosarcoma, representing less than 10% of primary malignant heart tumours (Burke and
Virmani, 1991), are mostly found at the left side of the heart (Burke et al. 1992) and in less than ten
cases in the right atrium or ventricle (Lurito et al. 2002).
Regardless whether the tumours are metastatic or primary the clinical picture resembles that of
chronic thromboembolism of the pulmonary artery with progressive dyspnoea, hypoxemia, high pul-
monary pressure, peripheral oedema, and congestive heart failure. Primary malignant heart tumours
behave aggressively, growing rapidly, and the prognosis of such malignant tumours is very poor (Burke
and Virmani, 1991; Burke et al. 1992; Van-der-Salm, 2000).
We report a case with primary osteosarcoma with base in the right ventricle occluding the pulmonary
outfl ow. The diagnosis was made late in the course of its development rendering it impossible for surgery.
Case
A man, 75 years-of-age, with a history of diabetes mellitus type II, asthma, and gout had surgery for
biliary stones four years ago. Last year he was referred to our hospital with progressive breathlessness,
infl ammation of the skin and peripheral oedema in both ankles. He had moderate renal failure with serum
creatinine concentration 1.6 mg/dl (149 µmol/l), corresponding to a glomerular fi ltration rate of 45 ml/min
(MDRD calculation) considered to be a consequence of diabetes mellitus nephropathy. A systolic cardiac
murmur grade 3/6 was found. The B-type pro-natriuretic peptide concentration was 7700 ng/l which in
spite of treatment with diuretics increased to 12200 ng/l while his body weight decreased by 6.5 kg.
Biochemical markers of ischemic disease, MB-fraction of creatine-phosphokinase and troponin-T, were
constantly slightly increased. C-reactive protein, haemoglobin, sodium, and potassium concentration
were normal. At electrocardiogram, low voltage, fi rst degree of atrial-ventricular block (PQ-time 0.28 ms),
Correspondence: Terje Forslund, Associate Professor, Department of Medicine, Central Finland Health Care
District Hospital, FIN-40620, Jyväskylä, Finland. Tel: +358 14 269 1812; Fax: +358 14 269 2711;
Email: terje.forslund@ksshp.fi or terje.forslund@med.uib.no
Copyright in this article, its metadata, and any supplementary data is held by its author or authors. It is published under the
Creative Commons Attribution By licence. For further information go to: http://creativecommons.org/licenses/by/3.0/.
Clinical Medicine: Oncology 2008:2 43–46
43

---

Forslund et al
some ventricular extra beats, and right bundle
branch block without signs of ischemic disease was
observed. The heart was only slightly enlarged with
no interstitial edema at chest x-ray examination.
Systolic and diastolic blood pressure ranged from
111/69 mmHg to 124/93 mmHg at arrival.
Cardiac ultrasound and doppler examination
(Fig. 1) disclosed a large tumour within the right
ventricle and atrium. Calcification was not
observed. The tumour almost totally occluded the
right ventricle outfl ow and pulmonary artery, and
blood fl ow was found in only a small edge of lumen
beside the tumour. The tricuspid leafl et was insuf-
fi cient with a regurgitate gradient of 100 mmHg Figure 2. Computerized scan tomography showing the large tumour
together with right atrial and ventricular dilatation. in the heart occupying the right atrial space and almost occlusion of
Computerized scan tomography (CT) and whole the pulmonary artery.
body CT examination (Fig. 2) verifi ed the intra-
ventricular tumour and excluded malignancy at
any other sites of the body. As contrast medium (Fig. 3). On sectioning the tumour was found to be
was used at CT examination calcifi cation could not gritty and partly calcifi ed. It had grown through the
be detected. Pulmonary scintigraphy examination pulmonary trunk into the pulmonary artery fi lling
excluded embolism. Surgical intervention at this almost the whole space of the right atrium (Fig. 3).
stage of the disease was considered impossible. Pericardial infi ltration and pericardial fl uid was absent.
His general condition worsened rapidly and he died The heart was otherwise normal with only moderate
two days later. Magnetic resonance imaging (MRI) to mild atherosclerosis of coronary arteries and with-
examination was not performed.
out ischemic areas. The liver was enlarged with signs
of right-sided congestion and oedema of the lungs was
Autopsy
present. No pathological changes were found in bone
structures or in any other organs of the body.
Macroscopy
Post-mortem autopsy disclosed a large heart (640 g) Microscopy
with a large gelatinous hard tumour based at the right The tumour was mostly composed of atypical
ventricular wall, 8 cm at length and 5–6 cm wide spindle cells with increased number of mitoses
(Fig. 4). Areas of new bone formation and osteoid
were readily found. New bone and osteoid were
produced by atypical tumour cells. Several areas of
necrosis could be seen. No other types of sarcoma-
tissues like chondrosarcoma, rhabdomyosarcoma,
angio-sarcoma or liposarcoma could be found. The
bone and osteoid forming tumour represented
primary osteosarcoma of the heart.
Discussion
In 1934 the fi rst clinical diagnosis of a primary
sarcoma of the heart, not osteosarcoma however,
was reported (Barnes et al. 1934). Much later the
fi rst reports on primary osteosarcoma-like tumour
of the heart containing a combination of tissues
and among them osteosarcomatous material
were published (Cumming and Shillitoe, 1957;
Figure 1. Echocardiogram showing the tumour in the right heart
occupying the whole right atrial area.
Hagström, 1961). In 1972 four cases of primary
44
Clinical Medicine: Oncology 2008:2

---

Primary osteosarcoma of the heart
malignant mesenchymoma in which additional
components of other sarcomatous material should
be found. Because of the location of primary osteo-
sarcoma in the right heart they may also be mis-
taken for atrial myxomas. In our case no such areas
or other types of sarcomas could be verifi ed.
The cellular mechanisms involved in the devel-
opment of primary osteosarcoma includes the
existence of a pluripotent precursor cell (mesen-
chymal stem cell) in the heart then to be activated
and transformed into active osteoblasts, a process
possibly infl uenced by i.e. PI3K-Akt-NFkappaB
pathways. Moreover, several genes seem to be
involved and may include over-expression of spe-
cifi c genes (i.e. TBHS3, ErbB2 protein). We were
Figure 3. Figure showing the opened heart showing the surface
not able to examine such mechanisms and we had
consisting of bony structures within the tumour.
certainly not any possibility to test any cytostatic
compounds or bisphosphonates prior to death in
bone-forming malignant heart tumours was our patient.
reviewed (Lowry and McKee, 1972). One previous
Neither ultrasound nor CT examination with
observation on primary osteogenic sarcoma of the contrast was able to distinguish calcifi cations.
left atrium has come from Sweden (Seidal et al. Whereas MRI examination would probably be the
1992), and one report on osteosarcoma from best method in order to discover intracardial
Finland included peripheral osteosarcomas with tumours CT examination without contrast should
secondary metastatic disease without any primary be preferred to distinguish calcifi cations within the
osteosarcoma of the heart (Elomaa et al. 1990). tumour. Because of the very fast growth of these
To our knowledge, this is the second case from tumours and since symptoms often occur late in
Scandinavia and the first case of primary the course, primary osteosarcoma of the heart is
osteosarcoma originating from the right ventricle almost always discovered late, the reasons why the
of the heart reported from Finland.
prognosis is very poor. Like in our case, the histo-
Cardiac neoplasms may arise from any portion logical diagnosis of the tumour is most often made
of the heart. While osteosarcomas metastatic to the post-mortem. In spite of increasing preciseness and
heart most commonly involve the right cardiac better technology only few patients with primary
chambers, most cases of primary osteosarcomas
were found in the left atrium (Burke et al. 1992).
Although primary osteosarcomas from the right
part of the heart have been reported (Murthy et al.
1976; Lurito et al. 2002) this anatomic occupation
is very unusual. The exact nuxmber of reported
cases of primary heart osteosarcoma is diffi cult to
defi ne. Only 32 cases of primary osteosarcoma of
the heart had been reported up to the year 2000
(Minami et al. 2000), and 20 out of 27 reported
cases had osteosarcoma at the left atrial location
in 2002 (Lurito et al. 2002). The broad base of
attachment would primarily have suggested meta-
static osteogenic sarcoma (Mich et al. 1985), and
the location of the tumour in our patient should
lead to suspicion of a possible metastatic process.
However, osteosarcoma could not be found in any
bone structures or anywhere else in the body. Dif-
Figure 4. Microscopic examination disclosed bony structures (oste-
oid) within the tumour with no other mesenchyme structures present
ferential diagnosis might include the possibility of pointing to osteosarcoma (van Gieson; magnitude x 40).
Clinical Medicine: Oncology 2008:2
45

---

Forslund et al
osteosarcoma are discovered before death. Once Rodríguez-Cruz, E., Cintrón-Maldonado, R.M. and Bercu, B.A. 1999.
suspected, the diagnosis is rapidly made and con-
Primary cardiac osteogenic sarcoma treated with heart transplantation.
Bol. Asoc. Med. P. R., 91:98–99.
sequently surgical intervention can be performed. Seidal, T., Wandt, B. and Lundin, S-E. 1992. Primary chondroblastic osteo-
Still, the prognosis remains very poor and deaths
genic sarcoma of the left atrium. Case report. Scand J. Thorac
from distant metastatic disease are common.
Cardiovasc Surg., 26:233–236.
Unfortunately this is also true after heart transplan-
Shuhaiber, J., Cabrera, J. and Nemeh, H. 2007. Treatment of a case of
primary osteosarcoma of the left heart: a case report. Heart Surg.
tation for malignant heart tumours with primary
Forum, 10:E30–32.
good outcome (Rodríguez-Cruz et al. 1999). Silverman, N.A. 1980. Primary cardiac tumors. Ann. Surg., 191:127–138.
Recently, successful surgery was reported in a Somers, K. and Lothe, F. 1960. Primary lymphosarcoma of the heart: Review
of the literature and report of 3 cases. Cancer, 13:449–457.
pregnant woman with low-grade osteosarcoma Torstveit, J.R., Bennett, W.A., Hinchcliffe, W.A. and Cornell, W.P. 1977.
(Koçac et al. 2006) and in another report more than
Primary plasmacytoma of the atrium. Report of a case with successful
one year survival (Shuhaiber et al. 2007) has been
surgical management. J. Thorac Cardiovasc. Surg., 74:563–566.
Vander Salm, T.J. 2000. Unusual primary tumors of the heart. Semin. Tho-
observed. Tumour resection and treatment with
rac Cardiovasc Surg., 12:89–100.
cytostatic compounds seems to achieve only tem-
porary improvement (Silverman, 1980; Burke and
Virmani, 1991; Van-der-Salm, 2000; Lurito et al.
2002). Surgical intervention seems justifi ed in
young subjects and in certain cases exemplifi ed in
that of a pregnant woman thereby saving a newborn
child (Koçac et al. 2006). Our patient was in a very
poor physical condition and consequently surgery
was considered to be no option.
References
Barnes, A.R., Beaver, D.C. and Snell, A.M. 1934. Primary sarcoma of the
heart: report of a case with electrocardiographic and pathological
studies. Am. Heart J., 9:480.
Bloor, C.M. 1978. Tumors of the endocardium and myocardium. In: Bloor
CM, (ed.) Cardiac Pathology. Philadelphia, J. Lippincott Co,
391–410.
Burke, A.P. and Virmani, R. 1991. Osteosarcomas of the heart. Am. J. Surg.
Pathol., 15:289–295.
Burke, A.P., Cowan, D. and Virmani, R. 1992. Primary Sarcomas of the
heart. Cancer, 69:387–395.
Cumming, ARR. and Shillitoe, A.J. 1957. Ball-valve mitral obstruction by
a sarcoma of a pulmonary vein. Br. Heart J., 19:287–289.
Elomaa, I., Siimes, M.A., Blomqvist, C. et al. 1990. Ten years’ experience
in patients with osteogenic sarcoma in Finland. Eur. J. Surg. Oncol.,
16:147–152.
Hagström, L. 1961. Malignant mesenchymoma in pulmonary artery and
right ventricle. Acta. Pathol., 51:87–94.
Janigan, D.T., Husain, A. and Robinson, N.A. 1986. Cardiac angiosarcomas:
a review and a case report. Cancer, 57:852–859.
Koçac, H., Karapolat, S., Gründoğdu, C. et al. 2006. Primary cardiac osteo-
sarcoma in a pregnant woman. Heart Vessels, 21:56–58.
Lowry, W.B. and McKee, E.E. 1972. Primary osteosarcoma of the heart.
Cancer, 30:1068–1073.
Lurito, K.J., Martin, T. and Cordes, T. 2002. Right atrial primary cardiac
osteosarcoma. Pediatr Cardiol, 23:462–465.
Mich, R.J., Gillam, L.D. and Weyman, A.E. 1985. Osteogenic sarcomas
mimicking left atrial myxomas: clinical and two-dimensional echo-
cardiography features. J. Am. Coll. Cardiol., 6:1422–1427.
Minami, H., Wakita, N., Kawanishi, Y. et al. 2000. Primary osteosarcoma
of heart with severe congestive heart failure. Jpn J. Thorac
Cardiovasc. Surg., 48:607–609.
Murthy, M.S., Meckstroth, C.V., Merkle, B.H. et al. 1976. Primary intimal
sarcoma of pulmonary valve and trunk with osteogenic sarcomatous
elements. Report of a case considered to be pulmonary embolus.
Arch. Pathol. Lab. Med., 100:649–651.
46
Clinical Medicine: Oncology 2008:2

---