brain metastases and multiple cerebral aneurysms from cardiac myxoma: case report and review of the...

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893 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. 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 calcification, located in both parietal and left frontal lobes. 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 calcification, 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. e right parietal lesion was resected entirely, in order to demonstrate the diagnosis and because of its mass effect. 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. e decision was to resect the left parietal calci- fied tumour in order to prevent the seizures. 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 deficits. 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 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. 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. 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 five cerebral microaneurysms. Keywords: cardiac myxoma; brain metastases; multiple intracranial aneurysms; surgery Br J Neurosurg Downloaded from informahealthcare.com by SUNY State University of New York at Stony Brook on 10/27/14 For personal use only.

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Page 1: Brain metastases and multiple cerebral aneurysms from cardiac myxoma: case report and review of the literature

893

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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Page 2: Brain metastases and multiple cerebral aneurysms from cardiac myxoma: case report and review of the literature

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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Page 3: Brain metastases and multiple cerebral aneurysms from cardiac myxoma: case report and review of the literature

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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