Download Four Cardiac Myxomas Diagnosed Three Times in One Patient

Eur J Echocardiography (2003) 4, 336–338
doi:10.1016/S1525-2167(03)00018-0
Four Cardiac Myxomas Diagnosed
Three Times in One Patient
K. Hermans1, W. Jaarsma1, H. W. M. Plokker1,
M. J. M. Cramer1 and W. J. Morshuis2
1
Department of Cardiology, Heart Lung Center Utrecht, St. Antonius Hospital,
Koekoekslaan 1, 3435 CM Nieuwegein, The Netherlands; and 2Department of Thoracic Surgery,
Heart Lung Center Utrecht, St. Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, The Netherlands
(Eur J Echocardiography 2003; 4: 336–338)
Ó 2003 The European Society of Cardiology. Published by
Elsevier Ltd. All rights reserved.
Introduction
revealed an atherosclerotic plaque with thrombus
formation. In December 1986 he was re-admitted for
a new TIA with right-sided hemiparesis. Oral anticoagulation therapy was started. An echocardiographic study showed a tumour in the left atrium
(LA), inserted on the border of the roof of the LA
and the interatrial septum (IAS). The mass was
surgically removed with patch closure of the discontinuity in January 1987. Histological examination
revealed myxoma cordis. Resection borders were free
of tumour tissue. Five months later, a routine control
echocardiography was performed. A new mass in the
right ventricular outflow tract of 3 3 cm inserted on
the IAS was seen. The mass was surgically removed.
The histological examination revealed cardiac myxoma and the resection borders were free of tumour
tissue. Echocardiographic control studies without
significant abnormalities were performed yearly until
1995. In December 2001, the patient returned at the
age of 72 years for a preoperative check for orthopaedic surgery. He was free of complaints but clinical
examination revealed a mitral regurgitation murmur.
The suspected mitral valve insufficiency (grades II/
IV) was confirmed by echocardiography but in addition, two tumours were detected, one inserted on the
atrial side of the anterior leaflet of the mitral valve
with prolapse through the mitral valve and the other
in the left ventricular cavity (Fig. 1). A conservative
approach was chosen because of the combination of
severe chronic obstructive pulmonary disease and the
Myxoma cordis is the most frequently found primary
cardiac tumour. A first recurrence of cardiac myxoma
has often been described. However, a second recurrence is rare. The incidence of recurrence is different
for the various types of myxoma cordis. Complex and
familial forms have a higher incidence of recurrence.
Surgical resection is the therapy of choice. In this
case report, we present a patient who experienced
four cardiac myxomas diagnosed over a period of 14
years.
Case Report
A 56-year-old man with known chronic obstructive
pulmonary disease was admitted for a transient
ischeamic attack (TIA) with right-sided hemiparesis
in October 1985. In 1986 he underwent surgical
embolectomy for acute lower limb ischaemia twice.
Histological examination was performed twice and
Address correspondence to: Dr. Kurt Hermans, Department
of Cardiology, Middelheim Hospital, P. Pourbusstraat 5,
2000 Antwerpen, Belgium. Tel: +32-476285973. E-mail:
[email protected]
Received 17 October 2002; revised manuscript received 5 March
2003; accepted 13 March 2003.
1525-2167/03/ $30.00/0
Key Words: myxoma cordis; recidive; surgical resection.
Ó 2003 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.
Downloaded from http://ehjcimaging.oxfordjournals.org/ at Pennsylvania State University on March 3, 2014
A left atrial myxoma was surgically removed in a 58-yearold man following several embolic events. Five months later,
a new myxoma was found in the right ventricular outflow
tract and surgically removed. In this patient, we visualized
two more recurrences 14 years later, one in the left atrium
and the other in the left ventricle. A short review of the
literature concerning recurrent cardiac myxomas is given.
Four Cardiac Myxomas Diagnosed Three Times in One Patient
337
unwillingness of the patient to undergo a third
operation. Treatment with oral anticoagulant therapy
was continued.
Discussion
Myxoma cordis is the most frequent primary heart
tumour to be found in humans. Its relative incidence
in combined surgical and pathological series is around
42%. When only surgical series is considered, the
proportion of myxomas increases to a relative incidence around 77%[1].
The tumour is a neoplasm of endocardial origin.
It consists of stromal cells arising from mesenchymal
multipotential cells, which are capable of neural and
endothelial differentiation[2]. Although cardiac myxomas are histologically benign, they may be lethal
because of their strategic position[3].
Of all myxomas 10% are familial forms. They are
diagnosed more frequently in younger patients and
appear to have an autosomal dominant transmission[4]. A complex myxoma occurs in families and
is associated with endocrine tumours, myxomas of
breast and skin, neurofibromatosis and spotty pigmentation. One of these associations is the Carney
Complex. It is linked with abnormalities on chromosome 2[5,6].
Recurrent cardiac myxoma after surgical excision is
not rare. The frequency is estimated by 1–3% in
sporadic forms, 12% in familial forms and 22% in
complex forms[7]. Second recurrence, however, is rare.
Only few cases of a second myxoma recurrence are
published so far[8,9]. The re-occurrence of myxoma can
be explained by several mechanisms. First, incomplete
resection of the original tumour can lead to regrowth.
Second, recurrence seems to have a familial predisposition. Third, intracardiac implantation of embolic fragments of the first tumour is possible. A
fourth possible explanation for recurrence on the
same or another location is the existence of a sort of
Ôpretumoural focusÕ in the myocardium. The resection
borders in our patient were two times free of tumour
tissue suggesting complete excision. The left and
right locations make intracardiac embolization of
the first tumour unlikely. None of the family members have cardiac myxomas or suspected signs of
familial cardiac myxoma. Therefore, we think that
this patient has a form of myocardial Ôpretumoural
tissue fociÕ.
The location of myxomas in the general population
is as follows: 75% in the LA, 23% in the right atrium
and only 2% in the ventricle. In 50% of the familial
forms multiple locations are present and around 10%
has a ventricular myxoma[10]. Careful inspection of all
heart chambers is necessary since multiple tumour
locations can be present, even if one is thrilled and
satisfied by a single spectacular finding.
Recidives are often asymptomatic and are mostly
detected by echocardiographic follow up studies.
Eur J Echocardiography, Vol. 4, issue 4, December 2003
Downloaded from http://ehjcimaging.oxfordjournals.org/ at Pennsylvania State University on March 3, 2014
Figure 1. Apical axis view: mass in LA protruding through mitral valve.
338 K. Hermans et al.
Eur J Echocardiography, Vol. 4, issue 4, December 2003
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Semiannual echocardiographic follow up studies are
therefore indicated for all cases[11]. The symptomatology in recurrent myxomas is the same as in general
non-recurrent myxomas. Location, size and mobility
are determinants of the clinical features. One or more
symptoms of the typical triad (embolism, obstructed
ventricular filling, general symptoms) can be present.
Small tumour particles or thrombus formation on
the tumour surface can explain the embolic events
that occur in 30–40% of myxoma patients[12,13]. No
tumour fragments were found in the histological
examinations following acute lower limb ischaemia in
our patient. Furthermore, no more embolic events
occurred after the start of anticoagulation suggesting
the tumour surface to be thrombogenic. It is our
opinion that long-term anticoagulation is warranted
in inoperable patients to prevent thrombo-embolic
events. Myxomas often give signs of obstructed
ventricular filling, mimicking thereby a mitral or
tricuspid valve stenosis. Production and release of
interleukin 6 by the tumour cells give rise to aspecific
symptoms such as fatigue, weight loss, fever and
arthralgia.
The speed of tumour growth can be calculated by
echocardiographic studies or by the interval between
the first and the second operations. It is estimated
that myxomas grow 0.15 mm in a month or 18 mm in
a year. The same calculations made for tumour mass
give a growth of 1.2 g in a month or 14 g in a year[14].
The first recurrence in this case (30 30 mm), not
visualized earlier, occurred after 5 months. Thus, in
this particular patient a growth of 6 mm in a month
can be estimated for the first recurrence.
The standard therapy for cardiac myxomas and
recurrent myxomas is surgical resection. Chemotherapy and radiation therapy are disappointing. Radical
excisional therapy and total cardiac transplantation
have been reported[15]. The role of orthotopic heart
transplantation remains unclear, even in malignant
heart tumours[16]. In our opinion, anticoagulation
therapy is warranted to prevent thrombo-embolic
events in inoperative patients.