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|Year : 2011 | Volume
| Issue : 3 | Page : 214-217
A rare diagnosis of multiple hemorrhagic metastases in brain
A Kumar, CE Deopujari, VS Karmarkar
Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India
|Date of Submission||08-Oct-2010|
|Date of Decision||11-Feb-2011|
|Date of Acceptance||27-Feb-2011|
|Date of Web Publication||22-Sep-2011|
Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
A 30-year-old female presented with an episode of generalized tonic-clonic convulsions. The imaging revealed multiple hemorrhagic lesions in the brain. There was no significant past history except for a cardiac surgery two years ago, for a benign atrial myxoma. The diagnosis of probable metastases was thought of and a thorough workup was planned, to determine the primary. Surprisingly, no primary lesion was detected, and therefore, as a histological diagnosis was required, she underwent navigation-guided excisional biopsy of one of the lesions. The histopathology proved that it was a myxomatous lesion. Thus, a rare diagnosis of atrial myxoma presenting as hemorrhagic metastases of the brain was formed. Atrial myxoma is a benign tumor of the heart. It can embolize distally and can present with known embolic/ischemic manifestations. However, presentation as hemorrhagic mass lesions, involving multiple areas of the brain, after many years of complete surgical excision, is quite rare and is rarely considered as a differential diagnosis. Hence, such a possibility should be kept in mind and careful primary cardiac intervention, with preventive measures to minimize the chances of distal embolization should be undertaken. As only few such cases have been reported worldwide, no treatment protocol has been devised at present, and a close follow-up of these patients is warranted.
Keywords: Hemorrhage, metastasis, myxoma
|How to cite this article:|
Kumar A, Deopujari C E, Karmarkar V S. A rare diagnosis of multiple hemorrhagic metastases in brain. J Postgrad Med 2011;57:214-7
| :: Introduction|| |
Primary cardiac tumors are a rare entity. Among them, atrial myxomas are the most common ones. They can be sporadic (93% of cases) or can occur in association with the Carney complex. Complete surgical removal of the myxoma and its cardiac attachment is usually curative. Recurrence has been related to incomplete excision, multifocality, and an embolism of tumor fragments. Neurological involvement can occur secondarily due to embolism into the central nervous system and thus, ischemic presentation forms the most common mode of presentation. Very rarely, a myxoma presents as a mass lesion and is termed as a 'metastasis'. Only isolated case reports are available in the literature and it is rarely thought of as one of the differentials. We report a case of a young female who presented to us with multiple hemorrhagic lesions in the brain. Presentation, imaging characteristics, and management of the case, along with relevant literature are discussed. This is only the second case report from India, and thus mandates awareness as an etiology, to form a management strategy for such patients.
| :: Case Report|| |
A 30-year-old female was admitted to our hospital with complaints of one episode of transient loss of memory followed by generalized tonic-clonic convulsions. On examination, she did not have any deficits except for papilloedema. Her computed tomography (CT) scan revealed multiple hemorrhages in different regions of the brain [Figure 1]. Magnetic resonance imaging (MRI) showed multiple hypointense lesions suggestive of fresh hemorrhage on T2 weighted images, along with blooming, on gradient echo sequences [Figure 2]. Most bleeds were acute and early subacute according to the MRI criteria. There was no contrast enhancement seen. She was operated for left atrial myxoma two years ago, when an echocardiogram detected a 5Χ5 cm-sized myxoma in the left atrium, with an ejection fraction of 60%. Surgical excision was done and she remained asymptomatic for two years till her current presentation. She was not taking any anti-inflammatory or anti-platelet drugs and her coagulation profile was normal. A repeat echocardiogram did not reveal any recurrent myxoma this time. Keeping metastasis in mind, a positron emission tomography (PET) scan was done, which revealed no primary. The erythrocyte sedimentation rate (ESR) and the tumor marker profile were within the normal range. As there was a need to establish tissue diagnosis, a navigation-guided excisional biopsy of the left frontal lesion was done. Intraoperatively, it was firm and well-defined [Figure 3]. The histopathology was consistent with myxomatous lesions, involving various areas of the brain with areas of aneurysmal dilatation of blood vessels [Figure 4]. A diagnosis of myxoma metastasis was made.
|Figure 1: Plain computed tomography scan showing multiple areas of hemorrhage in different lobes|
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|Figure 4: Matrix rich in acid mucopolysaccharides along with aneurysmal dilatation of vessels (H and E staining, original magnification ×200)|
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| :: Discussion|| |
Atrial Myxomas are thought to arise from multipotential mesenchymal cells of the endocardium (Pucci et al. ). They are one of the most common and benign tumors of the heart. They are usually sporadic, but can occur in association with spotty pigmentation (blue nevi), Cushing syndrome, acromegaly, testicular tumors, and schwannomas (Carney complex).
Patients with atrial myxomas usually present in three ways: (a) embolic manifestations, (b) cardiac failure, (c) systemic involvement in the form of fever, weight loss, high ESR, and abnormal serum proteins (hypergammaglobunemia) (Greenwood;  Peters et al. ). Systemic embolism of myxoma fragments has been described well in literature, the incidence being 45% with involvement of the brain in half of them (Greenwood;  Peters et al. ). Although, presentation as mass lesions (when they are termed as metastasis) is rare, and only few reports are available in literature. Desousa et al. in a review article had proposed a working classification of myxoma metastasis, in 1978:
- Metastasis, intraluminal only - (a) symptomatic (infarct); (b) asymptomatic
- Metastasis with vessel wall invasion - (a) occlusion of vessel, gradual; (b) aneurysm formation
- Metastasis with vessel wall transgression - (a) asymptomatic; (b) mass lesion effect
- Local cardiac recurrence - (a) at the original site; (b) at a different site
They observed that out of the 19 patients who had symptomatic arterial occlusion, 15 had cerebral emboli and four had extracranial emboli. According to Ashalatha et al.,  neurological involvement occurs due to various mechanisms such as ischemic stroke, with focal deficits or hemiparesis as the chief presentation. There can be intraparenchymal hemorrhage secondary to aneurysmal rupture. They can present with progressive dementia due to multiple subcortical infarctions, or lastly they can present as metastatic deposits in cerebral parenchyma or leptomeninges, although this is particularly rare, and our case is one of them.
Vascular transgression leading to the formation of intra-axial metastasis has been described by Bazin et al. The first case report of myxoma metastasizing to the brain, presenting as a mass lesion was published in journal CHEST, in 1978, by Rankin and Desousa,  while intracerebral hemorrhage possibly due to aneurysmal rupture was first reported by Price et al. Areas of involvement were lentiform nucleus and internal capsule, with intraventricular and subarachnoid extension. The first case report from India of an atrial myxoma presenting with intracerebral hemorrhage, due to rupture of these 'myxomatous' aneurysms, was reported by Ashalatha et al., in 2005. The primary cardiac tumor was excised six months ago and the patient presented with focal seizures with secondary generalization. Furuya et al. and Salyer et al. have given hypotheses like neovascularization of the vasa vasora and abnormal reparative processes of the vascular thrombi, respectively, for the aneurysmal formation, although the exact pathogenesis still remains unknown.
Awareness regarding this mode of presentation (without any evidence of stroke) is needed so that it forms one of the differentials for multiple hemorrhages in the brain, if there is any previous suggestive history. Usually if any young patient presents with such multifocal hemorrhages in the brain, after ruling out hypertension and bleeding disorders, hemorrhagic metastases (choriocarcinoma, melanoma, and renal and thyroid carcinoma) are suspected. Even though we were aware of the history of myxoma excision in the past, we never thought that cerebral myxoma metastases could present in this manner, and thus, after the workup for the primary was negative, we had to biopsy the lesion for histological diagnosis. There is no definite guideline for management of these lesions, as their natural history is unknown. Some patients have undergone 50 Gy whole brain radiotherapy. Some have been given chemotherapy (Doxorubicin/Ifosfamide). These are fusiform aneurysms precluding clipping. However, as per literature, aneurysm excision or wrapping has been done in the past for moderate-sized aneurysms.  If the masses are one or two, they can be excised surgically. However, such possibilities are rare and thus strict surveillance and following these patients is the only feasible option. Observation with repeated imaging may be needed in most of the cases, and only then can treatment protocols be standardized.
This is only the second published case from India and the patient will undergo angiography in next follow-up, to identify any myxomatous aneurysms, as the same was not done during the present admission due to non-affordability. The patient has been started on long-term anticonvulsants and is being monitored closely.
Thus, late cerebral metastasis after complete excision of the myxoma and its attachment is a rare phenomenon and can present as multiple hemorrhagic mass lesions, without any focal deficit. Usually surgery renders a complete cure, but tumor handling during cardiac surgery may cause microembolization of the myxomatous tissue to the nervous system, which may manifest as mass lesions, many years after the initial presentation. As the natural history of these lesions is unknown, the standard of cure for these intraparenchymal lesions remains unestablished. Thus, a high index of suspicion is required so that diagnosis can be effectively made and patients can be put under surveillance.
| :: References|| |
|1.||Pucci A, Gagliardotto P, Zanini C, Pansini S, di Summa M, Mollo F. Histopathological and clinical characterization of cardiac myxoma: Review of 35 cases from a single institution. Am Heart J 2000;140:134-8. |
|2.||Greenwood WF. Profile of atrial myxoma. Am J Cardiol 1968;21:367-75. |
|3.||Peters MN, Hall RJ, Cooley DA, Leachman RD, Garcia E. The clinical syndrome of atrial myxoma. JAMA 1974;230:695-700. |
|4.||Desousa AL, Muller J, Campbell R, Batnitzky S, Rankin L. Atrial myxoma: A review of the neurological complications, metastases, and recurrences. J Neurol Neurosurg Psychiatry 1978;41:1119-24. |
|5.||Ashalatha R, Moosa A, Gupta AK, Krishna Manohar SR, Sandhyamani S. Cerebral aneurysms in atrial myxoma: A delayed, rare manifestation. Neurol India 2005;53:216-8. |
|6.||Bazin A, Peruzzi P, Baudrillard JC, Pluot M, Rousseaux P. Cardiac myxoma with cerebral metastases. Neurochirurgie 1987;33:487-9. |
|7.||Rankin LI, Desousa AL. Metastatic atrial myxoma presenting as intracranial mass. Chest 1978;74:451-2. |
|8.||Price DL, Harris JL, New PF, Cantu RC. Cardiac myxoma: A clinicopathologic and angiographic study. Arch Neurol 1970;23:558-67. |
|9.||Furuya K, Sasaki T, Yoshimoto Y, Fujimaki T, Kirino T. Histologically verified aneurysm formation secondary to embolism from cardiac myxoma. Case report. J Neurosurg 1995;83:170-3. |
|10.||Salyer WR, Page DL, Hutchins GM. The development of cardiac myxomas and papillary endocardial lesions from mural thrombus. Am Heart J 1975;89:4-17. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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