An aggressive cervical hemangioma involving the vertebral artery: a case report

Introduction

Primary cervical vertebral tumors are rare, contributing to 10% of the overall incidence of all cervical vertebral tumors, secondary metastatic ones, however, are very common (1). These primary masses can arise from virtually any tissue in the cervical region, which include various histological entities such as osteoid osteomas, osteoblastomas, giant cell tumors, aneurysmal bone cysts, and vertebral hemangiomas (2,3). Vertebral hemangiomas are by far the most common masses that arise from the vertebral column; especially the cervical region (2).

Hemangiomas are benign masses that arise from vascular proliferation (4). These masses can be categorized into two types, either capillary or cavernous (5). The capillary subtype is superficial and is commonly in children, while cavernous hemangiomas are deep and are found in adults (5). Vascular anomalies, including hemangiomas, are often mistaken with Arterio-venous malformations. Therefore, the International Society for the Study of Vascular Anomalies (ISSVA) developed a classification system (6). The main difference the ISSVA concluded was that in tumors (hemangiomas), histological examination reveals hyperplasia, while in other anomalies such as arterio-venous malformation, it will reveal hypertrophy (6).

Although rare, hemangiomas originating from the cervical vertebrae can occur (7). The vast majority of these lesions are asymptomatic and are incidentally detected during imaging, while 5% of patients develop symptoms (8,9). When symptomatic, these masses typically evolve into an aggressive soft tissue mass, known as aggressive vertebral hemangioma (AVH), invading adjacent structures, leading to nerve compression, disturbed blood flow, and radiculopathy (10,11). Imaging of these masses can also be classified as typical or atypical (12,13). In typical cases, axial computed tomography (CT) imaging commonly demonstrates the “polka dot” sign, characterized by small, punctate areas of high attenuation from hyperdense trabeculae surrounded by hypodense stroma (14). On axial magnetic resonance imaging (MRI), these lesions present as a hyperintense mass with a central hypo intensity, which mirrors the polka dot sign seen on CT (13). Atypical hemangiomas, however, are often mistaken with metastatic malignancies due to their hypo- to isointense appearance on MRI (14). Microscopic analysis of these lesions reveals a characteristics thin-walled blood vessels and sinusoidal channels between prominent bony trabeculae (14). As the lesion progresses, the vascular component becomes more pronounced, contributing to the hypo intensity seen in atypical vertebral hemangiomas (14). For asymptomatic patients or those with mild to moderate symptoms, conservative management with observation remains the primary treatment approach (15). Surgical management is considered for patients presenting with significant radiculopathy (15). Vertebral hemangiomas recurrence rates are considered very low to non-existent, especially after total resection of the mass (11,16).

Surgical intervention of such tumors carries a high risk of mortality and morbidity, particularly if preformed in a staged approach for aggressive large tumors. Therefore, we propose that the risk can be minimized if a single approach with preoperative endovascular embolization is utilized. This is a report of a rare and complex case of an AVH at the C3 level, resulting in nerve root compression on the right side from C2 to C4. The tumor extended to encase the right vertebral artery (VA). This case was effectively managed through a multidisciplinary approach, involving collaboration between the spine surgery, otolaryngology, and interventional radiology (IR) teams, ensuring both the safety and efficacy of the treatment approach. We present this case in accordance with the CARE reporting checklist (available at https://jss.amegroups.com/article/view/10.21037/jss-25-80/rc).

Case presentation

A 34-year-old non-smoker male, who is medically and surgically free, was referred to King Faisal Specialist Hospital and Research Centre (KFSHRC) with a 3-year history of chronic, progressively worsening cervical pain accompanied by occasional occipital headaches. His Visual Analog Scale (VAS) score for pain was 8/10. Motor and sensory neurological examinations revealed no abnormalities, with no clinical evidence of myelopathy or radiculopathy. The patient didn’t report any history of trauma or neck surgery.

Initial investigations were carried out in the King Fahad Specialist Hospital in Dammam, Saudi Arabia, cervical spine X-rays revealed a lytic lesion and a mild reduction of disc space between C3–C4 and C4–C5 with loss or cervical lordosis (Figure 1). CT scan showed scalloping and erosions of foramen transversarium of C2, C3 and C4 vertebrae. The scalloped margins generally appeared corticated, more suggestive of pressure erosion than bone destruction (Figure 2). The lesion was centered on the nerve root exit foramina which appeared expanded. CT Angiography showed that the lesion completely encases the whole circumference of encasing the distal part of the V2 of the right VA. Codominant vertebral arteries and hypoplastic posterior communicating arteries were also noted. MRI showed lobulated right-sided hyperintense mass extending from C2 down to C4 involving the right side of the body and pedicle of C2, right side of the body, transverse process, and pedicle of the C3 and C4. The lesion was involving the C3 vertebral body and the posterior elements on the right side, causing pressure erosion on the right lateral side of C2 vertebral body (around 40% of the vertebral body) and C4 vertebral bodies (Figure 3). A CT guided biopsy was performed and it was suggestive of aggressive vascular hemangioma.

Figure 1 Preoperative posteroanterior and lateral radiographs of the cervical spine revealed a mild disc space narrowing, most prominent at the C3–C4 levels and to a lesser extent at C4–C5 and C5–C6.

Figure 2 Preoperative mid-sagittal and axial CT scans showed bony scalloping and erosion of the right side of the C2-C3-C4 cervical vertebrae with scalloped margins that appear to be corticated. The lesion was centered on the nerve root exit foramina which appear expanded. Erosion of the right-sided foramen transversarium was noted at these three vertebral levels. CT, computed tomography.

Figure 3 Preoperative sagittal STIR and T2 and axial MRI showed a well-defined lobulated hyperintense lesion within the neural foramina of right C2–C3 and C3–C4 measuring 4 cm × 4.5 cm × 4.2 cm in maximum anterior-posterior, transverse and craniocaudal dimensions. The mass demonstrated intraspinal extension with epidural component. MRI, magnetic resonance imaging; STIR, Short Tau Inversion Recovery.

Following a thorough discussion with the patient regarding the management possibilities of such tumor, and the fact that only surgical treatment could be a valid option, a decision was made to perform an occlusion test of the right VA by the IR team, with the potential for embolization. The patient was admitted one day prior to the procedure. Informed consent was obtained by the IR team for a head and neck angiogram, balloon occlusion test, and possible embolization.

An initial diagnostic angiogram revealed codominant vertebral arteries and showed the tumor to be hypovascular with no clear feeder and causing mild compression of the right VA. Under full heparinization and a blood pressure of 158/84 mmHg, the IR team initiated the occlusion test, maintaining right VA occlusion for 30 minutes. Clinical neuromonitoring in the awake patient was well tolerated without any decline of neurological status. Following a successful test, a multidisciplinary decision was made to continue with endovascular occlusion of the right VA in preparation for surgical resection. Twenty detachable three-dimensional (3D) coils with diameters ranging from 12–4 mm and lengths from 8–30 cm (Mix of Medtronic Axium and Stryker target coils) were deployed in the right VA, from the distal V3 segment above the C1 level down to the C5 level, achieving complete occlusion. The patient neurological status remained unchanged following permanent occlusion, and there was no evidence of stroke.

On the following day, informed consent was obtained from the patient, later he was brought to the operation room (OR). The surgery was later conducted in collaboration with the Otolaryngology (ENT) team, whom began the operation by conducting a wide surgical approach and right-sided neck dissection. The dissection was from the upper part of C2 and extended to the lower part of C5. Once the dissection was done, spinal team confirmed the C3 vertebral level with intraoperative fluoroscopy. Following confirmation, a C3 corpectomy was performed and a tissue from the tumor was taken and sent for fresh frozen sampling. The patient had night neck pain, and increased use of narcotics. Thus, complete tumor resection with corpectomy was performed to ensure postoperative pain relief.

Decompression of the spinal canal was done, allowing complete visualization of the dura mater without any residual compression. Further dissection of the tumor was performed on the right side, extending into the musculature beneath the longus colli. The entirety of the tumor was completely excised via the anterior approach including its lateral and posterior extension, which was facilitated by the prior coiling of the VA. The excised tumor and specimens were later sent for histopathological analysis. The tumor was revealed to have increased vascularity associated with inflammation, suggestive of a hemangioma. A mesh cage was placed at the level of C3 and was secured with anterior dynamic compression plate spanning from C2 to C4, which was affixed with four screws confirmation of correct placement of the construct was done with intraoperative imaging. Hemostasis was achieved, and the surgical site was thoroughly irrigated. A drain was placed, and the surgical wound was closed using standard techniques. The operative time was 5 hours and 27 minutes with an estimated blood loss of 400 milliliters.

After surgery, the patient was transferred to the Intensive Care Unit (ICU) for a 24-hour long observation. The ENT service was consulted postoperatively as the patient developed dysphagia and hoarseness. No intervention was needed, and the symptoms resolved completely within 3 weeks. The patient was discharged following the surgery one week later.

In follow-up visits, the patient showed stable progress with no recurrence of symptoms. His neck pain improved significantly with an VAS score of 2/10. Neurological examination demonstrated preserved motor and sensory function. Postoperative imaging showed a satisfactory cervical spine alignment, with the mesh cage appropriately positioned and the anterior plate and screws from C2 to C4 intact, also were the hardware-related complications (Figure 4). Additionally, stable therapeutic coil occlusion of the right VA was observed (Figure 5). MRI was performed immediately following the surgery and subsequently at 6 months, 1 year, and 2 years postoperatively to monitor for potential recurrence. The most recent MRI at the 2-year follow-up showed no tumor recurrence (Figures 6,7). All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Figure 4 Postoperative posteroanterior and lateral radiographs of the cervical spine showed an intervertebral cage occupying the C3 body, a spanning anterior plate from C2 to C4, and embolization coils deployed within the right vertebral artery.

Figure 5 Postoperative mid-sagittal and axial CT scan views of the C2, C3 and C4 levels showed stable tumor resection and C3 corpectomy with a mesh cage and anterior plate placement transfixing C2 to C4. CT, computed tomography.

Figure 6 Postoperative sagittal STIR and T2 MRI views at 2-year follow-up showed no tumor recurrence. MRI, magnetic resonance imaging; STIR, Short Tau Inversion Reconvery.

Figure 7 Postoperative axial T2 MRI views of the C2, C3 and C4 at 2-year follow-up showed no tumor recurrence. MRI, magnetic resonance imaging.

Discussion

Vertebral hemangiomas, first described by Virchow in 1867 and identified as benign vascular lesions by Perman in 1926 (4), are vascular spaces lined by endothelial cells (4). While these lesions are often found within the osseous borders of the vertebra, they rarely extend and compress nearby structures (4). Overexpression of various growth factors such as vascular endothelial growth factor (VEGF) and fibroblast growth factors (FGFs) are the main reason behind these lesions (3,5). Patients can live for decades without noticing the hemangioma, as these lesions are often asymptomatic and are found incidentally (8,9). As the lesion starts to expand and compress structures such as the neural bud, symptoms arise such as pain, myelopathy, and nerve compression (14,15). The mainstay treatment for asymptomatic patients and those with mild to moderate symptoms is conservative observation (15). Surgical resection and decompression are offered to patients with uncontrolled symptoms (15).

Although rare, cervical vertebral tumors can directly involve or encase the VA (16). The management of such tumors typically follows one of two primary strategies: either sacrificing and resecting the VA en bloc with the tumor, or preserving the artery through careful skeletonization (17).

Westbroek et al. proposed a structured algorithm to guide surgical decision-making in these cases, incorporating three key factors—tumor pathology, extent of VA encasement, and the redundancy of the posterior circulation. This system, referred to as the oncologic-mechanical-vascular (OMV) framework, helps determine whether VA sacrifice or preservation is more appropriate (18). In cases of primary tumors or isolated metastatic lesions where en bloc resection is indicated, VA sacrifice is generally recommended. From a morphological standpoint, skeletonization is typically considered feasible if the tumor encases less than 180 degrees of the VA circumference. Conversely, if encasement exceeds 180 degrees or if angioinvasion is present, complete resection often requires VA sacrifice. Additionally, specific vascular anatomical factors may contraindicate VA sacrifice, including bilateral VA encasement, absence of a contralateral VA, lack of a vertebrobasilar junction, or the presence of a patent segmental radiculomedullary artery originating from the affected segment (18).

Molina-Martínez et al. [2021] described a challenging case of a 40-year-old woman with a C3–C4 cervical chordoma that invaded the right VA. Their approach involved a multi-stage, circumferential technique to achieve maximal tumor resection while preserving the VA. Initially, a minimally invasive cervical laminectomy was performed to partially resect the tumor, followed by an anterior cervical approach for C3 and C4 corpectomies, addressing the tumor’s invasion into the posterior longitudinal ligament and dura. The final stage included a posterior approach to achieve complete tumor removal, with careful management of the VA (19). Although the staged approach avoided ischemia or VA sacrifice, our case proposed a different strategy. By performing a preoperative embolization, a single stage tumor resection was possible, reducing both surgical complexity and duration. This approach minimized the overall operative time and enhanced recovery by reducing the need for multiple surgeries.

By utilizing 3D printing, Liu et al. [2022] was able to employ a novel two-stage surgical approach in the treatment of a giant AVH involving the C2 to C4 vertebral bodies. The first stage involved partial resection of the AVH through a posterior approach, and decompression fixation at C1 to C6. The second stage involved an anterior cervical corpectomy and fusion, with a personalized 3D-printed implant (20). While Liu et al. [2022] incorporated 3D printing to assist him and his team in removing the mass in multiple stages, our approach involved a preoperative endovascular embolization, which facilitated the removal of the tumor in a single stage.

In the presented case, the tumor demonstrated greater than 180 degrees of VA encasement, necessitating consideration of VA sacrifice. Given advancements in endovascular techniques, a preoperative occlusion test was performed to assess the patient’s tolerance for right VA sacrifice, with the option of subsequent endovascular embolization. The patient successfully tolerated the occlusion test, and as the right VA was determined to be non-dominant, endovascular coil embolization was performed, extending from the C1 to C5 levels. This preoperative endovascular intervention significantly reduced the potential morbidity and mortality associated with intraoperative VA sacrifice alone. Furthermore, the endovascular coiling facilitated complete tumor resection, including the posterior tumor component, through a single anterior approach—a technique not commonly described in the existing literature (19,20).

Although preoperative VA embolization can facilitate complete tumor resection, consideration should be taken in the presence of an underlying medical condition such as coagulopathy and chronic kidney disease as they increase the risk of intraoperative bleeding (21). Another consideration that should be accounted for in an unsatisfactory preoperative angiography as the feeder artery to the tumor can arise from a segment of the spinal artery, which is a contraindication of VA embolization (22,23).

Conclusions

Managing cervical spine tumors that involve the VA is not easy task—it’s a real surgical challenge. This case highlights the importance of a multidisciplinary team collaboration for the successful treatment of a particularly aggressive cervical vertebral hemangioma that had wrapped itself around the VA. It’s a rare and challenging situation.

Staged approach for such invasive tumors might be a valid one, especially when they extend into the posterior areas. However, a different strategy was adopted in the current case. Preoperative endovascular VA occlusion with a single-stage anterior resection was paired. By using coil embolization beforehand, complete removal of both the anterior and posterior elements of the tumor was safely performed, which allowed us to minimize the risks and complications of the surgical intervention.

Care should be taken while preforming the preoperative embolization and should be stopped if the patient has an underlying coagulopathy or preoperative VA anatomy is not known.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://jss.amegroups.com/article/view/10.21037/jss-25-80/rc

Peer Review File: Available at https://jss.amegroups.com/article/view/10.21037/jss-25-80/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jss.amegroups.com/article/view/10.21037/jss-25-80/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. This study was conducted in accordance with the ethical principles outlined in the Declaration of Helsinki. Informed consent was obtained from all participants prior to their inclusion in the study.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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