Case Report
Stroke Caused by a Myxoma Stenosing the Common Carotid Artery Elena Cortes-Vicente, MD,* Raquel Delgado-Mederos, PhD,* Sergi Bellmunt, MD,† Xavier F. Borras, MD,‡ Beatriz G omez-Ans on, MD,x Silvia Bague, MD,jj Pol Camps-Renom, MD,* and Joan Martı-Fabregas, PhD*
Background: We report a case of stroke due to stenosis caused by a myxoma in the common carotid artery with no evidence of a cardiac origin. Only 1 such case has been reported previously in the literature. Methods: A previously healthy 37-yearold woman presented with repeated episodes of acute focal deficits together with motor, sensory, and language symptoms typical of left internal carotid territory involvement. Brain magnetic resonance imaging showed acute and subacute ischemic lesions in the territory of the left middle cerebral artery and border zone infarcts (middle cerebral artery with anterior and posterior cerebral arteries). Magnetic resonance angiography showed a filling defect in the distal portion of the left common carotid artery causing stenosis over 70%. Transesophageal echocardiography showed no embolic sources. Blood tests ruled out a prothrombotic state. Results: The image was initially interpreted as a possible subacute thrombus and anticoagulation was started. No changes were observed in the follow-up carotid ultrasound examination after 12 days of treatment. A gelatinous mass was removed during carotid surgery. No subjacent lesion was observed in the vessel wall. Pathology examination showed a spindle cell fibromyxoid tissue with fibrinoid material typical of myxoma. Conclusions: We hypothesize that the myxoma originated in the vessel, or alternatively, that a cardiac myxoma embolized without leaving a residual cardiac tumor. Although exceptional, myxoma should be added to the list of unusual causes of carotid artery stenosis causing stroke. Key Words: Myxoma—carotid artery—carotid stenosis—stroke—ischemia. Ó 2015 by National Stroke Association
From the *Stroke Unit, Neurology Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona; †Vascular Surgery Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona; ‡Cardiology Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona; xNeuroradiology, Radiology Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona; and jjPathology Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Received July 29, 2014; revision received September 10, 2014; accepted October 31, 2014. Address correspondence to Elena Cortes Vicente, MD, Stroke Unit, Department of Neurology, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Mas Casanovas 90, 4th floor, Barcelona 08041, Spain. E-mail: [email protected]. 1052-3057/$ - see front matter Ó 2015 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2014.10.020
Journal of Stroke and Cerebrovascular Diseases, Vol. 24, No. 4 (April), 2015: pp e87-e89
e87
e88
E. CORTES-VICENTE ET AL.
Myxomas are the most common type of primary heart tumor. Myxomas embolize in 30%-40% of patients, affecting the cerebral arteries in most cases.1 However, a myxoma embolism with no evidence of cardiac tumor is uncommon.
Case Presentation A 37-year-old woman presented acute onset of language difficulties. In the previous 4 months, she had also complained of loss of sensation and weakness in the right arm. She had no medical history of note. Neurologic examination showed moderate global aphasia, right homonymous hemianopia, right-sided brachial mild weakness, and hemihypesthesia, scoring 6 in the National Institute of Health Stroke Scale. Brain magnetic resonance imaging showed acute and subacute ischemic lesions in the territory of the left middle cerebral artery, and in the border zones between the middle cerebral artery and the anterior and posterior cerebral arteries (Figs 1 and 2). Magnetic resonance angiography showed a filling defect in the distal portion of the left common carotid artery, close to the carotid bifurcation, causing a stenosis greater than 70%. No other extracranial or intracranial lesions were observed (Fig 3). Carotid duplex ultrasound examination showed an isoechogenic image in the left common carotid artery, producing a focal increase in peak systolic velocity up to 200 cm/second and retrograde flow, compatible with a greater than 70% stenosis.
Figure 1. Brain magnetic resonance imaging, diffusion sequence. Hyperintense areas in frontal and parietal lobes suggesting acute ischemic lesions.
Figure 2. Brain MRI, FLAIR sequence. Hyperintense areas in border-zone territory between the MCA and PCA, suggesting subacute ischemic lesions. Abbreviations: FLAIR, fluid-attenuated inversion recovery; MCA, middle cerebral artery; MRI, magnetic resonance imaging; PCA, posterior cerebral artery.
This image was initially interpreted as a thrombus so anticoagulation with intravenous heparin was started. A transesophageal echocardiography showed no structural
Figure 3. Magnetic resonance angiography: subocclusive filling defect in the left common carotid causing significant stenosis (.70%).
STROKE CAUSED BY A CAROTID MYXOMA
Figure 4. Carotid surgery: reddish gelatinous mass attached to the carotid. No arteriosclerosis or other vascular lesions were observed. Color version of figure available online.
cardiac or aortic embolic sources. Blood tests ruled out a prothrombotic state. A carotid duplex examination 12 days after the onset of anticoagulation showed no changes. Carotid surgery (Fig 4) was performed 15 days after admission. A reddish gelatinous mass attached to the vessel wall was found. Once removed, the arterial wall showed no underlying lesions. A spindle cell fibromyxoid tissue with fibrinoid material typical of myxoma was found in the pathologic study (Fig 5). Follow-up transesophageal echocardiography and a cardiac magnetic resonance imaging (4 and 10 months after the stroke) showed no evidence of cardiac myxoma.
Conclusions The interest of the present case is its uncommon etiology. This is the second case report of an ischemic stroke
e89
Figure 5. Pathologic study: spindle cells in a fibromyxoid stroma, histologic appearance typical of myxoma.
caused by a myxoma in the carotid artery without evidence of a cardiac origin.2 We hypothesize that the myxoma originated as a primary tumor in the vessel wall, although no cases of primary vascular myxoma have been reported to date. Another possibility is that a cardiac myxoma embolized without leaving any trace of a residual tumor. Therefore, myxoma, although exceptional, should be added to the list of unusual causes of carotid artery stenosis causing stroke.
References 1. Reynen K. Cardiac myxomas. N Engl J Med 1995; 333:1610-1617. 2. Robbin NA, Landless P, Cooper K, Fritz VU. Myxoma in the carotid artery. Myxomatous occlusion of internal carotid artery. Stroke 1997;28:456-458.
Stroke Caused by a Myxoma Stenosing the Common Carotid Artery Elena Cortes-Vicente, MD,* Raquel Delgado-Mederos, PhD,* Sergi Bellmunt, MD,† Xavier F. Borras, MD,‡ Beatriz G omez-Ans on, MD,x Silvia Bague, MD,jj Pol Camps-Renom, MD,* and Joan Martı-Fabregas, PhD*
Background: We report a case of stroke due to stenosis caused by a myxoma in the common carotid artery with no evidence of a cardiac origin. Only 1 such case has been reported previously in the literature. Methods: A previously healthy 37-yearold woman presented with repeated episodes of acute focal deficits together with motor, sensory, and language symptoms typical of left internal carotid territory involvement. Brain magnetic resonance imaging showed acute and subacute ischemic lesions in the territory of the left middle cerebral artery and border zone infarcts (middle cerebral artery with anterior and posterior cerebral arteries). Magnetic resonance angiography showed a filling defect in the distal portion of the left common carotid artery causing stenosis over 70%. Transesophageal echocardiography showed no embolic sources. Blood tests ruled out a prothrombotic state. Results: The image was initially interpreted as a possible subacute thrombus and anticoagulation was started. No changes were observed in the follow-up carotid ultrasound examination after 12 days of treatment. A gelatinous mass was removed during carotid surgery. No subjacent lesion was observed in the vessel wall. Pathology examination showed a spindle cell fibromyxoid tissue with fibrinoid material typical of myxoma. Conclusions: We hypothesize that the myxoma originated in the vessel, or alternatively, that a cardiac myxoma embolized without leaving a residual cardiac tumor. Although exceptional, myxoma should be added to the list of unusual causes of carotid artery stenosis causing stroke. Key Words: Myxoma—carotid artery—carotid stenosis—stroke—ischemia. Ó 2015 by National Stroke Association
From the *Stroke Unit, Neurology Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona; †Vascular Surgery Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona; ‡Cardiology Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona; xNeuroradiology, Radiology Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona; and jjPathology Department, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Received July 29, 2014; revision received September 10, 2014; accepted October 31, 2014. Address correspondence to Elena Cortes Vicente, MD, Stroke Unit, Department of Neurology, Biomedical Research Institute Sant Pau (IIB Sant Pau), Hospital de la Santa Creu i Sant Pau, Mas Casanovas 90, 4th floor, Barcelona 08041, Spain. E-mail: [email protected]. 1052-3057/$ - see front matter Ó 2015 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2014.10.020
Journal of Stroke and Cerebrovascular Diseases, Vol. 24, No. 4 (April), 2015: pp e87-e89
e87
e88
E. CORTES-VICENTE ET AL.
Myxomas are the most common type of primary heart tumor. Myxomas embolize in 30%-40% of patients, affecting the cerebral arteries in most cases.1 However, a myxoma embolism with no evidence of cardiac tumor is uncommon.
Case Presentation A 37-year-old woman presented acute onset of language difficulties. In the previous 4 months, she had also complained of loss of sensation and weakness in the right arm. She had no medical history of note. Neurologic examination showed moderate global aphasia, right homonymous hemianopia, right-sided brachial mild weakness, and hemihypesthesia, scoring 6 in the National Institute of Health Stroke Scale. Brain magnetic resonance imaging showed acute and subacute ischemic lesions in the territory of the left middle cerebral artery, and in the border zones between the middle cerebral artery and the anterior and posterior cerebral arteries (Figs 1 and 2). Magnetic resonance angiography showed a filling defect in the distal portion of the left common carotid artery, close to the carotid bifurcation, causing a stenosis greater than 70%. No other extracranial or intracranial lesions were observed (Fig 3). Carotid duplex ultrasound examination showed an isoechogenic image in the left common carotid artery, producing a focal increase in peak systolic velocity up to 200 cm/second and retrograde flow, compatible with a greater than 70% stenosis.
Figure 1. Brain magnetic resonance imaging, diffusion sequence. Hyperintense areas in frontal and parietal lobes suggesting acute ischemic lesions.
Figure 2. Brain MRI, FLAIR sequence. Hyperintense areas in border-zone territory between the MCA and PCA, suggesting subacute ischemic lesions. Abbreviations: FLAIR, fluid-attenuated inversion recovery; MCA, middle cerebral artery; MRI, magnetic resonance imaging; PCA, posterior cerebral artery.
This image was initially interpreted as a thrombus so anticoagulation with intravenous heparin was started. A transesophageal echocardiography showed no structural
Figure 3. Magnetic resonance angiography: subocclusive filling defect in the left common carotid causing significant stenosis (.70%).
STROKE CAUSED BY A CAROTID MYXOMA
Figure 4. Carotid surgery: reddish gelatinous mass attached to the carotid. No arteriosclerosis or other vascular lesions were observed. Color version of figure available online.
cardiac or aortic embolic sources. Blood tests ruled out a prothrombotic state. A carotid duplex examination 12 days after the onset of anticoagulation showed no changes. Carotid surgery (Fig 4) was performed 15 days after admission. A reddish gelatinous mass attached to the vessel wall was found. Once removed, the arterial wall showed no underlying lesions. A spindle cell fibromyxoid tissue with fibrinoid material typical of myxoma was found in the pathologic study (Fig 5). Follow-up transesophageal echocardiography and a cardiac magnetic resonance imaging (4 and 10 months after the stroke) showed no evidence of cardiac myxoma.
Conclusions The interest of the present case is its uncommon etiology. This is the second case report of an ischemic stroke
e89
Figure 5. Pathologic study: spindle cells in a fibromyxoid stroma, histologic appearance typical of myxoma.
caused by a myxoma in the carotid artery without evidence of a cardiac origin.2 We hypothesize that the myxoma originated as a primary tumor in the vessel wall, although no cases of primary vascular myxoma have been reported to date. Another possibility is that a cardiac myxoma embolized without leaving any trace of a residual tumor. Therefore, myxoma, although exceptional, should be added to the list of unusual causes of carotid artery stenosis causing stroke.
References 1. Reynen K. Cardiac myxomas. N Engl J Med 1995; 333:1610-1617. 2. Robbin NA, Landless P, Cooper K, Fritz VU. Myxoma in the carotid artery. Myxomatous occlusion of internal carotid artery. Stroke 1997;28:456-458.
