brain metastases and multiple cerebral aneurysms from cardiac myxoma: case report and review of the...
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British Journal of Neurosurgery, December 2012; 26(6): 893–895
© 2012 The Neurosurgical Foundation
ISSN: 0268-8697 print / ISSN 1360-046X online
DOI: 10.3109/02688697.2012.692841
Brain metastases and multiple cerebral aneurysms from cardiac myxoma: case report and review of the literature
Mugurel Petrinel Radoi , Florin Stefanescu & Dorel Arsene
National Institute of Neurology and Neurovascular Diseases – Bucharest, Neurosurgical Department, Bucharest, Romania
Correspondence: Mugurel Petrinel Radoi, MD, PhD, National Institute of Neurology and Neurovascular Diseases – Bucharest, Neurosurgical Department,
10 - 12 Berceni Str., 041902 Bucharest, Romania. Tel: � 40 - 723527292. E-mail: [email protected]
Received for publication 10 March 2012 ; accepted 7 May 2012
Introduction
Cardiac myxoma is frequently found in the left atrium.
Despite their predominantly benign features, cardiac myxo-
mas can metastasize to extracardiac sites by embolization of
tumour particles. Th ey can give rise to brain metastases and
cerebral vascular disorders, such as infarcts, haemorrhages
and intracranial aneurysms. 1 With the review of the litera-
ture, we reported a peculiar case of a patient, operated for a
left atrial myxoma, who simultaneously presented multiple
brain tumours, proved to be cerebral metastases of cardiac
myxoma by histopathologic studies and multiple cerebral
aneurysms on the peripheral branches of the main cerebral
arteries.
Case report
A 45-years-old man was admitted to our hospital in June
2010, complaining of headache, nausea, gait disturbances
and weakness of the left extremities. Two episodes of gener-
alized seizures were documented in the month prior to his
admission in the hospital. On neurological examination, he
showed left grade III hemiparesis and sensory abnormality,
left homonymous haemianopsia and alert mentality. His
medical history revealed an open heart surgery, for a left
atrial myxoma, in January 2009.
Magnetic resonance imaging (MRI) (Fig. 1A, B) revealed
three intracranial tumours, with calcifi cation, located in both
parietal and left frontal lobes. Th e largest tumour was the
right parietal one, with 71/48/52 cm. in diameters, and which
shifted midline with 10 mm. It had a solid portion, with het-
erogeneous gadolinium enhancement, with intratumoural
necrosis and calcifi cation, and was surrounded by important
cerebral oedema. Four vessels cerebral angiography (Fig. 1C)
showed multiple unruptured intracranial microaneurysms,
which were mainly located in the peripheral branches of the
left anterior and middle cerebral arteries.
Th e right parietal lesion was resected entirely, in order to
demonstrate the diagnosis and because of its mass eff ect. On
gross examination, it was an encapsulated tumour, contain-
ing cysts, necrosis and haemorrhage. Microscopic examina-
tion revealed a benign myxoma metastasis (Fig. 1D).
Five weeks after surgery, he was readmitted to the hospi-
tal with the symptoms of focal seizures located in the right
extremities. Th e decision was to resect the left parietal calci-
fi ed tumour in order to prevent the seizures. Th e same histo-
pathologic diagnosis was reported. His post-operative recov-
ery was slow but improving, and after 3 weeks from his last
surgery he left the hospital with minor neurological defi cits.
During the 12 months follow-up, control cerebral CT
scans showed no signs of recurrence of the resected tumours
and the remaining frontal lesion did not change in size.
Discussion
Th e histogenesis of cardiac myxoma is not completely clear
and the neoplastic nature of the tumour was previously
questioned. Malignant features of myxoma as increased
mitotic activity and hypercellularity have been reported and
it is not known yet whether cardiac myxoma metastases were
originally malignant or showed later malignant transfor-
mation. Th e incidence of systemic embolization of benign
cardiac myxomas varies between 30% and 50%. Beside
cerebral tissue, other sites such as, muscles, bones, skin, eye,
kidney and coronary arteries, can be involved.
Th e mechanism of aneurysm formation, in patients
with cardiac myxoma, is not very clearly determined, but
SHORT REPORT
Abstract
Cardiac myxoma, the most common benign cardiac tumour, can
determine brain metastases or multiple cerebral aneurysms,
but very few cases of both complications have been reported.
We discuss the therapeutic management in the case of a
patient, operated for a cardiac myxoma, who presented three
intracerebral tumours and fi ve cerebral microaneurysms.
Keywords: cardiac myxoma; brain metastases; multiple
intracranial aneurysms; surgery
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894 M. P. Radoi et al.
some theories have been proposed: (a) cerebral vessels are
infi ltrated by myxoma cells, via vasa vasorum, and, thus,
the architecture of arterial walls is destroyed similar to the
mechanism of mycotic aneurysms, (b) tumour cells produce
vascular occlusion and perivascular lesions with subse-
quent scarring and pseudoaneurysm formation, (c) tumour
cells produce destruction of arterial wall architecture and
subsequent aneurysm dilatation by direct transendothelial
invasion. 2 In our case, aneurysms formed without previous
symptoms of cerebral embolism. Our observation is sup-
ported by the fact that most aneurysms occurred without a
previous history of cerebrovascular embolization.
Brain metastases from cardiac myxoma are usually found
at the same time or few months after the diagnosis of pri-
mary lesions. Th ere were authors who reported metastatic
brain lesions earlier than the diagnosis of cardiac myxoma,
but a latency interval, up to 8 years from the diagnosis of
primary cardiac lesion, had also been reported. Other sys-
temic metastases of cardiac myxoma in kidney, pancreas
and stomach, concomitant with the cerebral ones, have been
reported. Th e most frequent association found seem to be
with the bone metastases. Th e particularity of the present
case was the simultaneous presence of brain metastases and
multiple intracranial microaneurysms, both caused by a left
atrial myxoma systemic embolization.
Due to rarity of the cases, there is no standard manage-
ment of patients with cerebral metastases from cardiac
myxomas. Although published case series showed that
immediate central nervous manifestation is often severe, a
good long-term prognosis has been reported. Most authors
suggested that surgery is appropriated for diagnostic purpose
in cases with one or two isolated brain metastases. 3 Pallia-
tive radiotherapy or chemotherapy could be administrated
to patients with multiple brain metastases in order to obtain
a longer period of time without recurrence. In the literature
review, we found two cases with multiple brain metastases
from cardiac myxoma that received radiotherapy or a com-
bination of post-operative radiotherapy and chemotherapy.
Severe cases of rapid growth of preexisting aneurysms, and
also, spontaneous thrombosis resulting in resolution of an
aneurysm, have been reported. Because of the persistent risk
of brain metastases and aneurysms formation, early diagno-
sis of cardiac myxoma is desirable. Surgery is appropriated in
cases with few isolated brain metastases or when one of the
lesion is life threatening.
Conclusions
In our case, surgery was necessary because one of the
tumours produced an important mass eff ect. We considered
that it is advisable to individualize the therapeutic strategy in
accordance with patient ’ s own prognostic factors. In selected
cases, the presence of multiple intracerebral microaneu-
rysms, in the absence of a subarachnoid or intracerebral
Fig. 1. (A, B) Pre-operative axial post-contrast T1-weighted MRI showing three intracerebral supratentorial lesions (arrows); the largest one had 71/48/52 cm in diameters, with a cystic component, enhanced gadolinium and produced a signifi cant mass eff ect; (C) lateral view of a four vessels digital subtraction angiography showing the presence of the aneurysms; most of the microaneurysms (arrows) were located in the peripheral braches of the left anterior and middle cerebral arteries; (D) general microscopic aspect of the tumour. Inset: Details of the stellate and spindle-shaped cells embedded in a loose eosinophilic matrix are obvious (Hematoxylin – Eosin, original magnifi cation 200 � ; inset � 400 � ).
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Brain metastases from cardiac myxoma 895
haemorrhages history, should not discourage an aggressive
surgical approach of the brain metastases, as it will prolong
overall survival interval and improve the quality of life.
Th en, a careful follow-up of known aneurysms or remaining
tumours is recommended.
Declaration of interest: Th e authors report no confl icts of
interest. Th e authors alone are responsible for the content
and writing of the paper.
References
DeSousa AL, Muller J, Campbell RL, Batnitzky S, Rankin L. Atrial 1. myxoma: a review of the neurological complications, metastases, and recurrences. J Neurol Neurosurg Psychiatry 1978;41:1119 – 24. Herbst M, Wattjes MP, Urbach H, Hutter-Inhetvin C, Becker D, 2. Klockgether Th , Hartmann A. Cerebral embolism from left atrial myxoma leading to cerebral and retinal aneurysms: a case report. AJNR 2005;26:666 – 9. Altadung MB, Ertas G, Ucer AR, Durmus S, Abanuz H, Calikoglu T, 3. et al . Brain metastasis of cardiac myxoma: case report and review of the literature. J Neurooncol 2005;75:181 – 4.
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