Multimodal treatment approach in a patient with multiple intracranial myxomatous aneurysms

David L. Penn Departments of Neurosurgery,

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Arianna B. Lanpher Departments of Neurosurgery,

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Jennifer M. Klein Departments of Neurosurgery,

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Harry P. W. Kozakewich Pathology, and

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Kristopher T. Kahle Departments of Neurosurgery,
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Cellular and Molecular Physiology, Yale University, New Haven, Connecticut

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Edward R. Smith Departments of Neurosurgery,

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Darren B. Orbach Radiology, Boston Children’s Hospital, Boston, Massachusetts; and

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The most common primary cardiac tumor is myxoma, typically originating in the left atrium. Emboli to the central nervous system can cause cerebral infarction or, rarely, seed tumor growth within vessel walls, causing myxomatous aneurysms. Fewer than 60 myxomatous aneurysms have been reported, including 2 cases in children. Here, the authors describe 2 different growing myxomatous aneurysms in a child successfully managed using a combined multidisciplinary approach. A 12-year-old boy developed a sudden headache, diplopia, gait instability, and speech difficulty. Magnetic resonance imaging revealed a left parietal hemorrhage and multifocal cerebral infarction, suspicious for an embolic etiology. A cardiac myxoma was identified in the left atrium and resected. Follow-up cranial vasculature imaging demonstrated multiple intracranial myxomatous aneurysms. These lesions were followed up, and serial imaging identified marked growth of 2 of them (right occipital and left parietal), prompting invasive intervention. The deep occipital lesion was better suited to endovascular treatment, while the superficial parietal lesion was amenable to resection. The patient underwent embolization of an enlarging fusiform aneurysm of the distal right posterior cerebral artery, followed by a left parietal craniotomy for a lesion of the distal left middle cerebral artery. Both procedures were performed without complications and achieved successful obliteration of the lesions, as confirmed by catheter angiography at the 30-month follow-up. To the authors’ knowledge, this report illustrates the first combined endovascular and open surgical treatment of 2 myxomatous aneurysms in a single patient. While acknowledging the rarity of this condition, this report illustrates the clinical manifestations and treatment challenges posed by myxoma and details a successful strategy that could be employed in similar scenarios.

ABBREVIATIONS

ICA = internal carotid artery; PCA = posterior cerebral artery.

The most common primary cardiac tumor is myxoma, typically originating in the left atrium. Emboli to the central nervous system can cause cerebral infarction or, rarely, seed tumor growth within vessel walls, causing myxomatous aneurysms. Fewer than 60 myxomatous aneurysms have been reported, including 2 cases in children. Here, the authors describe 2 different growing myxomatous aneurysms in a child successfully managed using a combined multidisciplinary approach. A 12-year-old boy developed a sudden headache, diplopia, gait instability, and speech difficulty. Magnetic resonance imaging revealed a left parietal hemorrhage and multifocal cerebral infarction, suspicious for an embolic etiology. A cardiac myxoma was identified in the left atrium and resected. Follow-up cranial vasculature imaging demonstrated multiple intracranial myxomatous aneurysms. These lesions were followed up, and serial imaging identified marked growth of 2 of them (right occipital and left parietal), prompting invasive intervention. The deep occipital lesion was better suited to endovascular treatment, while the superficial parietal lesion was amenable to resection. The patient underwent embolization of an enlarging fusiform aneurysm of the distal right posterior cerebral artery, followed by a left parietal craniotomy for a lesion of the distal left middle cerebral artery. Both procedures were performed without complications and achieved successful obliteration of the lesions, as confirmed by catheter angiography at the 30-month follow-up. To the authors’ knowledge, this report illustrates the first combined endovascular and open surgical treatment of 2 myxomatous aneurysms in a single patient. While acknowledging the rarity of this condition, this report illustrates the clinical manifestations and treatment challenges posed by myxoma and details a successful strategy that could be employed in similar scenarios.

Myxomas are benign tumors, comprising approximately half of all cases of primary cardiac tumors, with an incidence of 0.5 per million population per year.13,17 Approximately 75% of myxomas arise from the left atrium, at or near the interatrial septum at the border of the fossa ovalis membrane.13,17 They are usually irregular in shape, gelatinous in consistency, and pedunculated, allowing them to swing freely and to intermittently obstruct flow across the mitral valve. Myxomas are more often found in women, with a female/male ratio of 2:1, and frequently affect patients between the 3rd and 6th decades of life.15 Symptoms at presentation can include cardiac murmur, syncope, and dyspnea, with severity ranging from asymptomatic presentation to lethal cardiac dysfunction and heart failure. Additionally, fragments of the tumor or blood clots from its surface can embolize and cause symptoms of ischemia in various other organ systems. Many patients with cardiac myxomas present with components of the clinical triad of intracardiac obstruction, embolism, and constitutional symptoms. Complete resection of the lesion is generally considered to be curative.

Most relevant from a neurological and neurosurgical perspective is when atrial myxomas present with systemic embolization to the central nervous system, as is the case in 10%–45% of patients.9,15,21 Embolization of myxomatous tumor or clot can result in cerebral infarction, with manifestation of symptoms depending on the location of the infarct, including hemiparesis, aphasia, or visual field deficits. Of the patients with myxoma presenting with systemic embolization, 26%–45% demonstrate neurological symptoms.9,11,15,20

In addition to stroke, embolization of tumor cells to the intracranial vasculature can result in tumor cell seeding of the brain and its blood vessels.4 This seeding can result in intracranial tumor growth or compromise of the integrity of the arterial wall with subsequent myxomatous aneurysm formation. Classically, myxomatous aneurysms are fusiform in morphology and are located on distal branches of the cerebral vasculature.25 Two theories of how tumor infiltration causes aneurysm formation have been proposed. In 1966, Stoane et al. suggested that large myxomatous emboli result in obstruction and perivascular damage with scarring followed by subsequent pseudoaneurysm formation.22 Aneurysms are then observed after recanalization of the blood vessel. In contrast, New et al. demonstrated invasion of the arterial wall by embolic myxoma cells, causing vessel destruction and dilation.14 Subsequent studies confirmed the latter findings and also revealed destruction of the endothelium and tumor cells in the subintimal space.5

Intracranial myxomatous aneurysms are rare, with fewer than 60 cases reported;10,18 the literature regarding the management of these lesions is even sparser. Despite gross-total resection of the primary lesion, myxomatous aneurysms can continue to enlarge and pose a risk of subarachnoid hemorrhage. Some have proposed chemotherapy to prevent further lesion growth.3,16 Low-dose radiation therapy in conjunction with chemotherapy has proven to be a more encouraging method of conservative management of intraparenchymal metastases.2 Open surgical management remains a viable option since the majority of these lesions are located in distal branches. Finally, with continued improvements in technology and technique, endovascular methods are emerging as successful means of treating myxomatous aneurysms.

The present case report details the management of a pediatric patient presenting with intracranial emboli leading to the discovery of a left atrial myxoma and multiple myxomatous aneurysms. This case is the first to demonstrate a multimodal approach to the clinical management of these lesions, with 1 lesion treated via endovascular embolization and 1 lesion treated via open resection.

Case Report

History

The patient initially presented at 12 years old with a medical history significant for migraines, gastroesophageal reflux disease, apneic spells as an infant that had since resolved, bilateral myringotomies, tonsillectomy, and toxic synovitis of the left hip. On the day of admission, the patient had been at football practice when he experienced the sudden onset of a throbbing headache that gradually increased in severity. Subsequently, he developed diplopia, perioral numbness, ataxia, slurred speech, and an episode of emesis. There was no loss of consciousness, though he reported having experienced 6 months of intermittent dizziness. The family history was significant for early cardiac events, lower-extremity deep vein thrombosis, stroke, and a distant relative with brain aneurysm.

Examination and Radiological Studies

Initial studies included a noncontrast head CT demonstrating a small amount of left posterior parietal hemorrhage, as well as CTA and MRI of the brain revealing multiple small acute infarctions within the left thalamus, pons, and right cerebellum together with areas of chronic infarction in the centrum semiovale bilaterally and right parietal white matter lesions consistent with embolic events (Fig. 1). Out of concern for a possible cardiac thrombus, a heparin drip was started. Echocardiogram demonstrated a large atrial mass adherent to the posterior leaflet of the mitral valve, a finding confirmed on cardiac MRI, which showed a gadolinium-enhancing mass concerning for atrial myxoma. The patient was transferred to our center for further evaluation and management. Neurological examination was significant for dysarthric speech, diplopia in medial visual fields, mild right-sided esotropia, right nasolabial flattening with good activation, minimal weakness in the proximal right upper and lower extremities, and slight right-sided dysmetria.

FIG. 1.
FIG. 1.

Imaging studies obtained on initial presentation. Axial CT scan (A) demonstrating a small amount of left parietal hemorrhage and axial susceptibility weighted image (B) with blooming artifact in the left parietal region, consistent with hemorrhage. Diffusion-weighted images (C and D) demonstrating diffuse restriction in multiple regions throughout the brain. Note the punctate foci of diffusion restriction in the right cerebellum and right facial colliculus (arrow, C). Note a small focus in the left pons (arrow, D) and a focus in the left thalamus (E).

Operation

Emergent sternotomy for resection of the left atrial mass under temporary cardiopulmonary bypass was performed successfully and without complication, achieving gross-total resection of the large, pedunculated gelatinous mass occupying the entire left atrium, which was later confirmed radiographically.

Postoperative Course

Postoperatively, the intubated patient was transferred to the ICU for intensive respiratory support and hemodynamic monitoring. He was extubated on postoperative day 1. Physical examination was significant for diplopia in the medial visual fields and subtle right nasolabial fold flattening with good activation. The remainder of his hospitalization was uneventful, and he was discharged home on postoperative day 3 on aspirin (81 mg daily).

At the 3-month follow-up, the patient reported overall improvement; however, he was still experiencing intermittent stabbing headaches and speech difficulties. Physical examination revealed the resolution of previously noted deficits. Magnetic resonance imaging and MRA of the brain demonstrated 10–11 foci of sulcal/subarachnoid space enhancement in continuity with many of the distal blood vessels, including a subset of these lesions possessing sulcal FLAIR hyperintensity consistent with possible collateral blood flow or partial thrombus (Fig. 2). Magnetic resonance angiography was unremarkable. Given the patient’s history of atrial myxoma and embolization, as well as the lesions’ fusiform configuration, we presumed the lesions were myxomatous aneurysms. Diagnostic cerebral angiography was first performed 4 months after initial presentation and revealed numerous (> 10) areas of flame-shaped or fusiform dilation on the right internal carotid artery (ICA) injection, remaining opacified through the mid-venous phase. In particular, a sausage-like fusiform dilation of the right posterior cerebral artery (PCA) in the posterior parietal region was observed (Fig. 3). Repeat angiography demonstrated interval increase of this lesion with daughter dilation, as well as 2 new similar foci on the left ICA injection. These lesions were believed to be consistent with myxomatous aneurysms secondary to embolization of tumor cells. Based on these findings, the decision was made to proceed with endovascular and surgical management of these lesions.

FIG. 2.
FIG. 2.

Follow-up MRI studies obtained 3 months after initial presentation. Axial postcontrast MRI sequences demonstrating contrast enhancement (arrows) in the right (A) and left (B) parietal regions. Numerous similar appearing lesions noted throughout the cerebrum are not demonstrated. Axial T2 FLAIR images (C and D) depicting the same lesions from panels A and B. High signal intensity (arrows) demonstrates possible delayed blood flow or partial thrombus.

FIG. 3.
FIG. 3.

Diagnostic cerebral angiograms obtained 4 months after initial presentation and at 1 year. A: Lateral view of right ICA injection in the late arterial phase demonstrating numerous flame-shaped and fusiform dilatations (arrowheads) throughout the supratentorial distal vasculature. B and C: Lateral views of left ICA injection in the early venous phase. Note the lesion in the left parietal lobe noted on MRI in Fig. 2. There is a fusiform bilobed dilation in the distal left middle cerebral artery candelabra (circles). On repeat angiography, growth and opacification of the lesion were visible. D and E: Anteroposterior view of right and left vertebral artery injections, respectively, demonstrating fusiform dilation of the right superolateral occipital branch off the right PCA (arrowheads) and interval growth. F: Anteroposterior view of left vertebral artery injection post–Onyx embolization demonstrating obliteration of the myxomatous aneurysm via superselective catheterization of the right superolateral occipital branch.

Endovascular Treatment

One year after initial presentation, the patient underwent liquid embolic embolization (Onyx, ev3 Neurovascular) of the right PCA occipital fusiform myxomatous aneurysm. It was decided to treat this lesion endovascularly given the depth and increased invasiveness required for resection. Because of the distal location, small parent vessel, and fusiform morphology of the aneurysm, we determined that only a deconstructive approach with a liquid embolic was feasible. The right superolateral occipital parent branch was accessed with a microcatheter, and the aneurysm was successfully occluded with Onyx 18 (Figs. 3 and 4). Hospitalization was unremarkable, and the patient was discharged with plans to return for surgical management of the aneurysmal lesions in the left parietal region.

FIG. 4.
FIG. 4.

A and B: Superselective catheterization of the right superolateral occipital branch off the PCA, anteroposterior and working views, respectively. C: Intraoperative photograph revealing dilated and fusiform abnormality of the left distal M4 branch in the parietal lobe after sulcal dissection. The lesion was subsequently isolated using bipolar electrocautery and excised.

Microsurgical Treatment

One month after embolization of the right PCA myxomatous aneurysm, the patient underwent microsurgical intervention for the left parietal lesion given its proximity to the cortical surface and the fact that its poorly visualized and tiny parent vessel would be difficult to access via endovascular techniques. The patient was brought to the operating room and positioned supine with his head turned to the right in the Mayfield head holder. A left parietal craniotomy was performed, and the lesion was localized with the aid of the Medtronic Stealth Stereotactic Frameless Navigation system (Medtronic) and confirmed with Doppler ultrasound. Once the lesion location was identified, the sulcus harboring the vascular lesion was explored. Upon identification of the dilated fusiform vessel, bipolar electrocautery was used to isolate the lesion via coagulation of the immediate proximal and distal parent vasculature (Fig. 4). The parent vessel was sharply divided and the lesion was removed without complication. Immediate postoperative angiography confirmed successful removal of the left parietal lesion and stable occlusion of the right occipital lesion (Fig. 5). Postoperatively, the patient’s hospitalization was again unremarkable.

FIG. 5.
FIG. 5.

Intraoperative repeat diagnostic cerebral angiograms obtained during left parietal craniotomy for microsurgical resection of the left parietal myxomatous aneurysm. A: Lateral view of left ICA injection demonstrating obliteration of the lesion. B: Anteroposterior view of left vertebral artery injection demonstrating stable obliteration of the right parietal myxomatous aneurysm at 1 month.

Pathological Findings and Follow-Up

Histopathological examination of the resected specimen showed aneurysmal dilation of the arterial wall with loss of medial smooth muscle and elastic laminae, particularly the internal elastic lamina (Fig. 6). Within the accompanying mural myofibroblastic proliferation were clusters of tumoral cells surrounded by myxoid matrix. The tumoral cells were highlighted by cytoplasmic immunoreactivity for calretinin.26

FIG. 6.
FIG. 6.

Histopathological examination of the resected aneurysm. A: Longitudinal oblique section of the excised artery showing aneurysmal dilation and myofibroblastic intimal proliferation (right half of image). H & E, original magnification ×40. B: Progressive loss of internal elastic lamina (arrowheads) in the wall of the aneurysm. Miller’s elastin stain, original magnification ×40. C: Wall of aneurysm with tumoral cells surrounded by myxoid matrix. H & E, original magnification ×400. D: Tumoral cells in the wall of the aneurysm delineated by cytoplasmic immunoreactivity for calretinin. Original magnification ×600.

Follow-up angiography at 30 months demonstrated stable obliteration of the 2 dominant myxomatous aneurysms. Other, previously visualized submillimeter lesions remained unchanged, and no new lesions were identified. Magnetic resonance imaging and MRA performed 43 months after presentation demonstrated expected postprocedural changes and stability of the untreated myxomatous aneurysms. The patient is followed up with annual MRI (thin-cut postcontrast T1-weighted images and MRA being the most important sequences), with catheter angiography reserved for instances in which there is a change in the MRI appearance.

Discussion

In the present report, we detail the case of a 12-year-old boy presenting with symptoms of cerebral ischemia suspicious for emboli, who was diagnosed with a left atrial cardiac myxoma and subsequently found to have multiple arterial lesions consistent with myxomatous aneurysms. After resection of the cardiac myxoma on initial presentation, follow-up imaging demonstrated progression of 2 of these cerebral lesions. Those seen to be progressing were treated with endovascular Onyx embolization and craniotomy for ligation. This case is unique because it demonstrates a rare pathological entity and its successful treatment with multiple modalities, including endovascular and microsurgical techniques.

Myxomatous aneurysms can be found months to years after definitive treatment of the primary cardiac lesion and also pose a significant risk of rupture given their fragile histopathology.27 Case reports have documented patients presenting with various neurological deficits between 4 months to 25 years after treatment of the primary lesion.4,27 Given that the incidence and natural history of these lesions is not well established, screening of patients with known primary cardiac myxoma, especially those who present with embolic symptoms, is reasonable. Once the lesions are discovered, close radiographic follow-up for interval growth is warranted to determine if and when treatment is necessary.12,24 If neurological symptoms are present, we advocate screening via MRI and/or MRA on presentation with repeat imaging at 3 months. Lesions detected on these initial studies warrant closer follow-up with interval imaging at 3 months to confirm stability. Once the lesions are determined to be stable without clinical significance, it seems reasonable to increase interval imaging to 6 months or annually based on clinician judgment and lesion characteristics. Since these lesions can present years after the initial presentation, one should maintain a low level of suspicion for patients presenting with spontaneous intracranial hemorrhage and a history of atrial myxoma.

For the most part, given the paucity of data on the natural history of myxomatous aneurysms, conservative management has been the method of choice in treating these lesions. The primary reason for this therapeutic strategy is that many of these lesions have demonstrated stability on radiographic follow-up, and some have even been documented to exhibit spontaneous regression.6,8,18,23 In a meta-analysis, Zheng et al. reviewed 37 patients presenting with multiple cerebral myxomatous aneurysms,28 finding that 78.4% of these patients were managed conservatively. From this subgroup, 75.9% demonstrated stability or even involution of these lesions and 20.7% demonstrated enlargement, with mortality being 3.4%.

Although conservative management demonstrates encouraging outcomes, the question of how to manage enlarging myxomatous aneurysms is significant. Current literature on the treatment of myxomatous aneurysms describes several different medical and surgical options. Reports have shown both failure and success in using chemotherapeutic agents to curb enlargement of these intracranial lesions, including doxorubicin, etoposide, and carboplatin.3,16 Treatment of intraparenchymal metastatic myxomatous lesions with doxorubicin and ifosfamide, in addition to external beam radiation, has led to the disappearance of tumor, which may suggest a role for this modality in treating intracranial vascular lesions.2 More invasive modalities, including resection of these lesions, offer the potential for definitive treatment and diagnosis, albeit with increasing risk to the patient.7 Craniotomy for complete excision, clip ligation, or bypass are possible treatment options depending on the size, location, and morphology of the lesion.4,19 However, clip reconstruction of these lesions is unlikely to be successful because of the friable tissue at these sites. With its advances in technology and techniques, the endovascular modality is increasingly promising.1 However, as is the case for open surgical treatment, reconstructive therapy of these friable lesions is unlikely to be successful, and deconstructive occlusion with a liquid embolic is probably the best option for most cases.

One caveat to the treatment of myxomatous aneurysms using endovascular techniques is that there is no definitive management for the tumor cells that have invaded the blood vessel. This presents a risk for recurrence of the lesion or even further local invasion. However, if the aneurysm is successfully occluded, the risk of hemorrhage would be significantly lowered. Furthermore, with complete occlusion and a decreased blood supply to the diseased blood vessel, a nonviable environment for tumor growth is created, minimizing the risk of regrowth or local invasion. As previously mentioned, residual tumor cells left behind after endovascular embolization and in stable lesions could be treated with chemotherapeutic agents and radiotherapy; however, these treatments have only been used for intraparenchymal lesions with limited efficacy, and their effect on aneurysmal lesions is unknown.2,3,16 Regardless, these lesions are successfully managed conservatively with acceptable rates of stability and regression, so the ramifications of residual tumor cells seem minimal and adjuvant treatment may be unnecessary.28 Without having a true understanding of the natural history of this pathological entity, it is difficult to predict whether these lesions will remain stable; therefore, continued radiographic follow-up is necessary.

In determining the best treatment strategy, lesion location and morphology are important factors, especially when choosing between microsurgical and endovascular methods. In a literature review, Tamulevičiūtė et al. analyzed 25 cases of myxomatous aneurysms from 24 case reports.25 Their analysis revealed that the majority of myxomatous aneurysms occur in distal branches. Among these 25 cases, 4 aneurysms occurred on proximal vessels, including the ICA, the M1 segment, the basilar artery, and the P1 segment. The remaining aneurysms occurred beyond the M2, A2, and P2 segments of their respective parent vessels. This same study found that, with the exception of 1 case, all aneurysms were fusiform in shape. Given the limitations of current endovascular techniques, access to extremely distal lesions is unlikely to be feasible, and thus surgical ligation would be a more optimal treatment.

Acknowledgments

We thank Kristin Johnson for her artistic and technical expertise in preparing this manuscript.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study for the findings specified in this paper.

Author Contributions

Conception and design: Smith, Orbach. Acquisition of data: Penn, Lanpher, Klein, Kozakewich, Kahle, Orbach. Analysis and interpretation of data: Smith, Penn, Klein, Kahle, Orbach. Drafting the article: Penn, Lanpher, Klein, Kozakewich. Critically revising the article: Smith, Penn, Klein, Kozakewich, Kahle, Orbach. Reviewed submitted version of manuscript: Smith, Penn, Lanpher, Kozakewich, Kahle, Orbach. Approved the final version of the manuscript on behalf of all authors: Smith. Administrative/technical/material support: Smith, Penn, Lanpher, Klein. Study supervision: Smith.

Supplemental Information

Previous Presentations

Portions of this work were presented in abstract form as a video poster at The New England Neurosurgical Society Annual Meeting 2017 held in Chatham, Massachusetts, on June 22–24, 2017.

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  • Imaging studies obtained on initial presentation. Axial CT scan (A) demonstrating a small amount of left parietal hemorrhage and axial susceptibility weighted image (B) with blooming artifact in the left parietal region, consistent with hemorrhage. Diffusion-weighted images (C and D) demonstrating diffuse restriction in multiple regions throughout the brain. Note the punctate foci of diffusion restriction in the right cerebellum and right facial colliculus (arrow, C). Note a small focus in the left pons (arrow, D) and a focus in the left thalamus (E).

  • Follow-up MRI studies obtained 3 months after initial presentation. Axial postcontrast MRI sequences demonstrating contrast enhancement (arrows) in the right (A) and left (B) parietal regions. Numerous similar appearing lesions noted throughout the cerebrum are not demonstrated. Axial T2 FLAIR images (C and D) depicting the same lesions from panels A and B. High signal intensity (arrows) demonstrates possible delayed blood flow or partial thrombus.

  • Diagnostic cerebral angiograms obtained 4 months after initial presentation and at 1 year. A: Lateral view of right ICA injection in the late arterial phase demonstrating numerous flame-shaped and fusiform dilatations (arrowheads) throughout the supratentorial distal vasculature. B and C: Lateral views of left ICA injection in the early venous phase. Note the lesion in the left parietal lobe noted on MRI in Fig. 2. There is a fusiform bilobed dilation in the distal left middle cerebral artery candelabra (circles). On repeat angiography, growth and opacification of the lesion were visible. D and E: Anteroposterior view of right and left vertebral artery injections, respectively, demonstrating fusiform dilation of the right superolateral occipital branch off the right PCA (arrowheads) and interval growth. F: Anteroposterior view of left vertebral artery injection post–Onyx embolization demonstrating obliteration of the myxomatous aneurysm via superselective catheterization of the right superolateral occipital branch.

  • A and B: Superselective catheterization of the right superolateral occipital branch off the PCA, anteroposterior and working views, respectively. C: Intraoperative photograph revealing dilated and fusiform abnormality of the left distal M4 branch in the parietal lobe after sulcal dissection. The lesion was subsequently isolated using bipolar electrocautery and excised.

  • Intraoperative repeat diagnostic cerebral angiograms obtained during left parietal craniotomy for microsurgical resection of the left parietal myxomatous aneurysm. A: Lateral view of left ICA injection demonstrating obliteration of the lesion. B: Anteroposterior view of left vertebral artery injection demonstrating stable obliteration of the right parietal myxomatous aneurysm at 1 month.

  • Histopathological examination of the resected aneurysm. A: Longitudinal oblique section of the excised artery showing aneurysmal dilation and myofibroblastic intimal proliferation (right half of image). H & E, original magnification ×40. B: Progressive loss of internal elastic lamina (arrowheads) in the wall of the aneurysm. Miller’s elastin stain, original magnification ×40. C: Wall of aneurysm with tumoral cells surrounded by myxoid matrix. H & E, original magnification ×400. D: Tumoral cells in the wall of the aneurysm delineated by cytoplasmic immunoreactivity for calretinin. Original magnification ×600.

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