Thematic review and illustrative videos of aggressive vertebral column hemangioma management: illustrative cases

Abstract

BACKGROUND

Aggressive vertebral hemangiomas (AVHs) are benign vascular lesions that can exhibit locally invasive behavior. Because of their rarity, standardized treatment guidelines are lacking. We present 2 surgically managed cases, supplemented with intraoperative video, and conduct a thematic literature review evaluating treatment strategies.

OBSERVATIONS

A 66-year-old female presented with low back pain radiating to the groin and gait instability. MRI demonstrated an L1 AVH. She underwent T12–L2 laminectomy, instrumented stabilization, and cement augmentation, followed by radiotherapy. Marked pain relief and functional improvement were reported. The second patient, a 14-year-old male, presented to his pediatrician with neck pain, hand weakness, and paresthesias. He sustained a fall at school and was brought to the emergency department with quadriparesis. Imaging revealed a C4–6 AVH with a C5 pathological fracture. Emergency C4–6 corpectomy, laminectomy, and posterior fixation were performed. Proximal upper extremity function partially recovered, but hand and lower extremity motor deficits persisted. A literature review demonstrated the importance of surgery and highlighted the roles of radiotherapy, cement augmentation, embolization, and alcohol-based injections in nonoperative and multimodal management.

LESSONS

AVH management should be tailored based on location, extent of neural compression, and symptom severity. Surgical intervention remains central in cases with neurological compromise or instability.

https://thejns.org/doi/10.3171/CASE25654

Vertebral hemangiomas are benign vascular proliferations in bone marrow consisting of adipocytes, blood vessels, and interstitial edema that contribute to vertical trabecular thickening. Vertebral hemangiomas have been identified in 11% of adults, although their prevalence is likely higher.^1,2 Most vertebral hemangiomas are clinically silent; however, approximately 1% of patients become symptomatic, and among these 20%–45% demonstrate aggressive features.^3,4

Aggressive vertebral hemangiomas (AVHs) are defined by their potential for rapid growth, cortical bone destruction, and extension into adjacent soft tissues. These features can lead to neurological compromise through compression of the spinal cord or nerve roots.⁵ Characteristic aggressive features include rapid proliferation, extension past the vertebral body, cortex disruption, and expansion into the neural arch, surrounding soft tissue, epidural space, and/or paravertebral space.^5,6

There is no current consensus on optimal AVH management. In this study, we present 2 surgically managed cases, 1 pediatric and 1 adult, to illustrate AVH treatment considerations. Narrated intraoperative videos detail surgical considerations and techniques for each case. A thematic literature review was performed using publications identified in the PubMed database. Search keywords included “aggressive vertebral hemangioma,” “symptomatic vertebral hemangioma,” “aggressive vertebral hemangioma surgery,” “aggressive vertebral hemangioma radiotherapy,” “aggressive vertebral hemangioma cement augmentation,” “aggressive vertebral hemangioma embolization,” and “aggressive vertebral hemangioma ethanol injection.” Reference lists of studies were also reviewed. Studies were included if they discussed AVH or symptomatic vertebral hemangioma management. Findings were grouped and analyzed according to modality.

Illustrative Cases

Case 1

A 66-year-old female with type 2 diabetes and osteoarthritis presented with 2 weeks of shooting, burning, and pressure-like low back pain radiating bilaterally to the groin with associated nausea. She initially underwent an outpatient CT study demonstrating an osseous hemangioma at L1 with possible thecal sac compression. The outpatient MRI study demonstrated a T1 and T2 hyperintense lesion at L1 containing regions of striated hypointensity extending into the pedicles and transverse processes bilaterally. Homogeneous signal loss was revealed on STIR and fat-saturated T1-weighted images. The lesion extended through the posterior vertebral cortex into the ventral epidural space, resulting in spinal canal stenosis. The patient was told to present to the emergency department.

In the emergency department, MR images were reviewed, and additional imaging was performed. Lumbar spine CT revealed moderate spinal stenosis from a predominantly hypodense lesion with thick hyperdense striations at the L1 vertebral body, suggestive of an AVH. There was no associated acute fracture. A CT study of the chest, abdomen, and pelvis ruled out malignancy. The patient denied balance trouble, bowel or bladder dysfunction, muscle weakness, or sensory changes. On neurological examination, there were no muscle weaknesses, sensory deficits, or upper motor neuron signs. After risks, benefits, and alternatives were discussed, the patient elected to proceed with resection with decompression and instrumentation, and informed consent was obtained. T12–L2 laminectomy, instrumented stabilization, and cement augmentation were performed (Video 1).

VIDEO 1. Separation surgery for management of an aggressive L1 hemangioma. Click here to view.

Frozen pathological analysis revealed a lesion within the intramedullary space and consisting of small fragments of crushed, bland, ovoid to spindled cellularity with interspersed vascular spaces. Examination also demonstrated trilineage hematopoiesis and a mild interstitial lymphoid infiltrate. These findings were consistent with a hemangioma.

Gross residual disease remained postoperatively, so the risks and benefits of further treatment with radiotherapy were discussed. Because of the aggressive lesion characteristics, the presence of pain postoperatively, and the complication risks associated with potential future interventions, the patient opted to receive adjuvant intensity-modulated radiation therapy (IMRT). IMRT was initiated 1 month postoperatively at a dose of 45 Gy across 25 fractions.

Postoperative, 3-month, 9-month, and 16-month MR images all demonstrated cement augmentation of L1, maintenance of vertebral height loss, and appropriate hardware placement with maintained alignment (Fig. 1). There was no residual compression of the spinal canal. Postoperative fluid collection in the resection bed had completely resolved on the 9-month MRI study. MRI also revealed no recurrence of aggressive features within the L1 vertebral body. Although the patient has previously exhibited degenerative scoliosis, radiographic imaging showed stable alignment and hardware (Fig. 1). The patient reported significantly reduced pain.

FIG. 1.

Preoperative and postoperative MR images and postoperative radiographs obtained in a 66-year-old female with a vertebral hemangioma. A: Preoperative sagittal postcontrast MR image showing enhancing lesion at L1. B: Postoperative sagittal postcontrast MR image demonstrating resection and vertebral body cement augmentation. C: Preoperative axial MR image illustrating the extent of the lesion. D:Postoperative axial MR image showing resolution of mass effect and cement within the vertebral body. E and F: Sagittal (E) and coronal (F) views showing T12–L2 posterior instrumentation with pedicle screws and rods, as well as vertebral body cement augmentation at L1.

Case 2

A 14-year-old male with no pertinent past medical history presented to his pediatrician with 3 months of neck pain, hand weakness, and distal upper extremity paresthesias.⁷ Radiographic imaging, MRI, and CT imaging were performed in the outpatient setting (Fig. 2). Radiographic imaging showed angular kyphotic deformity at C5 with right-sided coronal concavity. MRI demonstrated C4–6 enhancement of both the anterior and posterior spinal elements, spinal cord compression, and T2 signal change (Fig. 2). CT imaging revealed an osteolytic fracture at C5 resulting in localized subaxial kyphosis, with bone loss at the C4 and C6 vertebral bodies. He then experienced a fall at school and presented to the emergency department with acute neurological deterioration and severe neck pain.

FIG. 2.

Preoperative MR images obtained in a 14-year-old male. A: Sagittal postcontrast MR image with arrows indicating enhancement from C4 to C6 involving both anterior and posterior spinal elements. B:Sagittal noncontrast MR images highlighting the extent of the lesion. C: Axial noncontrast MR image showing marked spinal cord compression. D: Axial postcontrast MR image demonstrating enhancement of surrounding soft tissues and spinal cord compression. MRI findings are notable for C4–6 enhancement, significant spinal cord compression, and associated T2 hyperintensity suggestive of cord edema.

Neurological examination demonstrated deltoids, biceps, and triceps strength bilaterally of 3/5, 4/5, and 2/5, respectively. Bilateral strengths for the wrist flexors, wrist extensors, grip, interossei, and major lower extremity muscle groups were 0/5. Sensation from the upper chest to the feet, including the perianal area, was absent. There was complete areflexia in the upper extremities, lower extremities, and bulbocavernosus reflex.

A two-stage procedure involving corpectomy and laminectomy at C4–6 with posterior fixation was performed (Video 2). The approach in totality amounted to a three-level spondylectomy at C4–6. Because of destabilization from the lesion, the lower instrumented vertebral body was extended lower than is typically utilized for this cervicothoracic fusion. Additionally, to improve stability and minimize risk of instrumentation failure, a cobalt-chrome four-rod construct was placed from C2 to T1 with a transition to a dual-rod construct extending to T3.

VIDEO 2.Three-level vertebrectomy for resection of an aggressive cervical hemangioma. Click here to view.

Throughout postoperative week 1, the mean arterial pressure was kept above 85 mm Hg, with the patient showing no improvement on neurological examination. He was discharged to inpatient rehabilitation. Follow-up imaging demonstrated effective spinal canal decompression and restored alignment at C5 (Fig. 3). Pathological analysis revealed thin-walled vessels, bony trabeculae, and cortical erosions. These findings were consistent with a hemangioma.

FIG. 3.

Postoperative radiographs obtained in a 14-year-old male following three-level spondylectomy (C4–6) and cervicothoracic fusion. Sagittal (A) and coronal (B) views demonstrating the superior portion of the construct: a four-rod construct extending from C2 to T1 (which thereafter transitions to a dual-rod construct extending to T3). Because of extensive tumor involvement of the C4–6 posterior elements and resultant spinal destabilization, fixation was extended lower than is typically utilized for this cervicothoracic fusion. Lateral mass screws were omitted at the subaxial levels due to tumor involvement, and a two-to-one rod transition was implemented to enhance construct durability.

At the 10-month follow-up, neurological examination demonstrated some improved bilateral extremity function: bilateral strengths for the deltoids, biceps, triceps, and wrist flexors were 5/5, 4+/5, 3/5, and 2/5, respectively. However, functional hand use and lower extremity motility remained extremely limited. The wrist extensors, grip strength, interossei, and lower extremities remained 0/5, except for occasional left toe flexion (1/5). Sensation remained absent from the upper chest to the feet.

Informed Consent

The necessary informed consent was obtained in this study.

Discussion

Observations

There is not currently consensus on optimal treatment for AVH. Nonoperative therapies including cement augmentation, sclerotherapy, and embolization have received increased consideration due to intraoperative bleeding risks and the lower complication rates of nonoperative modalities.⁸ Nonetheless, treatment is frequently multimodal and often involves both surgery and nonoperative adjuncts.⁹ The roles of resection, radiotherapy, cement augmentation, embolization, and alcohol-based injections in an effective management protocol are described in this section and in a proposed treatment algorithm based on expert opinion and synthesis of current literature findings (Fig. 4). However, this algorithm has not been validated in a clinical setting. Key findings of the thematic literature review are described in Table 1.

FIG. 4.

Proposed algorithm for management of symptomatic AVH in adult patients that synthesizes expert opinion and current literature findings, but is not yet validated in a clinical setting.

TABLE 1.

Key findings of AVH management: thematic literature review

Management	Findings
Resection	Surgery is a well-established treatment option, particularly for patients w/ severe or rapidly progressing symptoms &/or high recurrence risk. Partial vs en bloc resection is debated & should be determined on a case-by-case basis. Periop hemostasis measures, including embolization, are critical to reduce intraop morbidity.
Radiotherapy	Stand-alone radiotherapy has demonstrated modest efficacy for patients w/ slowly progressive, painful AVH w/ minimal neurological deficits. Radiotherapy is often used as a postop adjuvant to reduce recurrence risk. Higher radiotherapy doses have been shown to be most effective, w/ SRS & IMRT enabling delivery w/o exceeding spinal cord tolerance.
Cement augmentation	Nonperiop cement augmentation has shown modest efficacy for treatment of slowly progressive, painful AVH w/ minimal neurological deficits. Frequently, cement augmentation is used perioperatively to reduce both recurrence risks & intraop blood loss.
Embolization	Embolization is an effective & commonly used periop hemostatic adjuvant.
Ethanol-based injections	Evidence supporting the use of stand-alone sclerotherapy & periop ethanol injections is limited.

Narrative Literature Review

Resection

Resection remains central to AVH treatment, with goals including decompression of neural elements and spinal stabilization.⁸ Surgery should be considered for patients presenting with severe or rapidly progressing symptoms.¹⁰ Early surgical intervention is important for treating patients with myelopathy or focal neurological deficits, as damage to neurological structures is difficult to reverse.⁷

AVH resection extent remains controversial, with prior studies supporting both partial and en bloc resection. Acosta et al. noted local recurrence in 3 of 10 patients with Enneking stage 3 vertebral hemangiomas following partial resection and thus concluded that partial resection may be associated with AVH relapse.¹¹ Similarly, Handa et al. concluded that the benefits of partial resection are temporary.¹² However, other studies have advocated for the use of partial resection due to the thought that decompression and stabilization are sufficient to relieve neurological symptoms, particularly when the tumor is in an anatomically challenging location.¹³ Collectively, these studies suggest that optimal resection extent is case dependent, and a tailored approach with consideration of anatomical constraints and risks for blood loss and recurrence should be used.

AVH size and location often influence optimal surgical technique. A posterior approach—either laminectomy or corpectomy—is typically used, unless the AVH is ventrally located, in which case an anterior approach may be required.³ Laminectomy is generally preferred in cases of spinal stenosis, as it results in less blood loss than corpectomy.⁸ Moreover, laminectomy with decompression and fusion is indicated for vertebral body compression fractures with neurological deficit.⁸ However, corpectomy and spondylectomy are the preferred techniques for patients with significant anterior column compromise and patients with extraosseous tumor expansion.^8,14 Instrumented stabilization is often used following laminectomy or corpectomy; however, instrumentation may be avoided in select thoracic cases with minimal instability.^7,8

Preventing intraoperative blood loss through embolization or cement augmentation is an important step to minimizing surgical morbidity. Robinson et al. advocated for the use of preoperative embolization, as blood loss was lower in patients receiving embolization prior to AVH surgery (980 ± 683 mL) than in patients who did not receive embolization (1629 ± 946 mL).¹⁵ Cement augmentation has also been proposed as an effective approach to reducing blood loss.^16,17 Given the vascularity of AVH, incorporation of a hemostatic modality is critical.

Retrospective studies demonstrate improved outcomes for patients with surgically managed AVH. In a 2020 meta-analysis of 197 surgically treated patients with neurological deficits, Piper et al. reported complete remission of symptoms in 84% of patients, although 8% of these patients subsequently experienced recurrence.¹⁸ In a meta-analysis of 535 surgically managed vertebral hemangiomas, Chopra et al. reported excellent Odom criteria outcomes in 81.7% cases.⁷ However, cases involving myelopathy or focal neurological deficits were less likely to be associated with excellent outcomes.⁷ Goldstein et al. reported a 3% local recurrence rate for surgically managed vertebral hemangiomas, although prior publications have identified recurrence rates as high as 27.3%.^13,19 These studies reinforce the utility of surgery in AVH management and highlight the importance of follow-up monitoring to identify recurrences in a timely manner.

Radiotherapy

Radiotherapy plays an important role in AVH treatment, including both as a stand-alone treatment in select cases and as an adjuvant to decompressive surgery. Radiotherapy is frequently used in the adjuvant setting to reduce AVH relapse risk.²⁰ The resulting vascular necrosis and anti-inflammatory effects from radiotherapy also reduce clinical symptom severity.⁸

AVH treatment with radiotherapy alone has demonstrated modest improvements in symptoms. Glanzmann et al. demonstrated that radiotherapy improved symptoms in 60% of a 62-patient cohort (only 7 of whom received surgery prior to radiotherapy).²¹ Similarly, Asthana et al. determined that all patients with pain and tenderness experienced full (87.5%) or partial (12.5%) responses, while those with numbness and paresis showed either complete (66.7%) or partial (33.3%) improvement following receipt of a 35- to 40-Gy dose.²² In this same study, 7 of 9 patients with paraplegia had full or partial recovery, while the 2 with longstanding paraplegia (> 6 months) had no response.²² More recently, Wang et al. reported a 65% long-term control rate of vertebral hemangiomas with mild or slowly progressive neurological deficits treated with primary radiotherapy at a dose of 40–50 Gy across 20–25 fractions.²³

While some studies demonstrated stand-alone radiotherapy efficacy in select cases, others instead supported radiotherapy as a surgical adjuvant. Of 10 patients treated with radiotherapy alone, Jiang et al. reported 2 patients with treatment failure.³ Because 3 of 6 patients receiving decompressive surgery alone also experienced recurrence, Jiang et al. advocated for radiotherapy following partial resection, as no recurrences occurred in patients receiving adjuvant radiotherapy.³ This study suggested improved AVH control with postoperative radiotherapy.

Radiotherapy is also central to recurrent AVH management. Surgery coupled with radiotherapy has been used to address symptomatic recurrences, while stand-alone radiotherapy is typically used in recurrences lacking neurological symptoms.^8,24 These findings suggest that radiotherapy alone can be considered in select patients lacking neurological deficits, in the treatment of both primary and recurrent lesions. However, relapse risks are likely greater compared to those of decompressive surgery with adjuvant radiotherapy.

Radiotherapy is most effective at higher doses, and newer technologies are enabling increased doses without exceeding spinal cord tolerance. Rades et al. demonstrated a dose-effect relationship and recommended a target radiation dose of 36–40 Gy, with a dose per fraction of 2.0 Gy.²⁵ However, neurological deficits can be exacerbated through radiotherapy, and therefore spinal cord tolerance constrains the maximum radiotherapy doses for conventional radiotherapy.²⁶ Aich et al. similarly concluded that the effect of radiotherapy on vertebral hemangiomas is dose dependent and highlighted IMRT use to deliver higher doses without exceeding spinal cord tolerance.²⁶

Recently, SRS has emerged as an alternative to conventional fractionated radiotherapy for AVH. Conti et al. reported complete, partial, and stable response rates of 45.7%, 23.6%, and 37.2%, respectively, across 23 patients with vertebral hemangiomas treated with SRS.²⁷ Similarly, Miszczyk et al. concluded in a randomized trial of 74 patients with painful vertebral hemangiomas that SRS delivered as 25 Gy over 5 fractions is a safe, significantly shorter, and potentially more effective technique compared with fractionated radiotherapy delivered at doses of up to 36 Gy.²⁸ These findings highlight the growing role of SRS in AVH treatment.

While radiotherapy plays a central role in adult AVH management, age-based considerations limit the use of radiotherapy in pediatric patients. Specifically, radiotherapy is often avoided in pediatric AVH treatment due to risks for growth plate damage and secondary malignancy.²⁹ Radiotherapy utilization is therefore a central difference in pediatric versus adult AVH management, as demonstrated through comparison of management of the 2 patients presented in this study.

Collectively, these studies reveal the importance of radiotherapy in adult AVH treatment, both as a stand-alone therapy and particularly as a postoperative adjuvant.

Cement Augmentation

Cement augmentation is a minimally invasive procedure involving injection of polymethylmethacrylate into the vertebral body to improve stability, relieve pain, and reduce bleeding and recurrence risks. It can be used alone for patients presenting exclusively with axial pain or in multimodal care.^10,30 While both kyphoplasty and vertebroplasty can be used for AVH, vertebroplasty is more frequently described in the literature. Vertebroplasty produces thrombosis and irreversible sclerosis of venous networks, resulting in AVH size reduction.⁸ Additionally, vertebroplasty consolidates trabecular microfractures and can improve patient mobility, spinal stability, and fracture-related back pain.⁸

Nonperioperative vertebroplasty can be considered as a treatment option for select patients lacking motor deficits. Premat et al. reported a mean visual analog scale score decrease from 7.23 ± 1.3 to 3.11 ± 1.9 across 26 AVH patients treated with percutaneous alcohol embolization and vertebroplasty.³¹ Moreover, 88% of the patients with neurosensory disorders had complete responses, although only 1 of 3 patients with motor deficits reported improvement.³¹ Therefore, this study demonstrated that alcohol embolization with vertebroplasty relieved pain and neurosensory symptoms but was less effective in addressing motor deficits.³¹ Roscop et al. similarly supported the efficacy of circumferential vertebroplasty in AVH pain management, with 9 patients completely responding, 11 partially responding, and 2 having no response at 3 months of follow-up.³² Outside the perioperative setting, vertebroplasty is most effective at alleviating AVH-related pain relative to other symptoms.

While select AVH cases may be managed nonoperatively with cement augmentation, it is more frequently used alongside decompressive surgery. In addition to reducing intraoperative blood loss, perioperative cement augmentation has been proposed as an approach to minimize AVH recurrence.¹⁶ Cement augmentation is therefore an effective adjuvant in multimodal AVH management.

Embolization

Transarterial embolization is commonly used preoperatively to minimize blood loss during AVH resection. The procedure involves occluding feeding arteries of the hemangioma, thereby reducing vascularity. Smith et al. identified 2 patients treated with embolization alone, neither of whom experienced clinical improvement.³³ Therefore, embolization is not typically utilized as a stand-alone treatment for AVH.³³ However, evidence supports preoperative embolization to reduce surgical morbidity, particularly intraoperative blood loss. Piper et al. reported reduced complications, improved clinical symptoms, and fewer recurrences in the 98 patients receiving preoperative embolization.¹⁸ Although not statistically significant, this trend and findings from other aforementioned studies supported preoperative embolization to reduce surgical morbidity.¹⁵

Ethanol-Based Injections

Both sclerotherapy and intraoperative ethanol injection are ethanol-based treatments for AVH, although their indications and risks differ. Sclerotherapy involves CT-guided direct injection of a sclerosing agent to induce thrombosis, shrink the lesion, and reduce pain. Of 11 patients with symptomatic vertebral hemangiomas treated with ethanol injection alone, Doppman et al. reported paraplegia resolution in 5 of 6 patients and radiculopathy improvement in 4 of 5 individuals.³⁴ However, the 2 patients receiving the largest doses (42 and 50 mL) both developed pathological vertebral fractures, leading the authors to advocate for a dose limit of 15 mL.³⁴ More recently, Premat et al. demonstrated that percutaneous alcohol embolization with vertebroplasty relieved pain and neurosensory symptoms but not motor deficits.³¹

Ethanol-based injections are also used perioperatively. Singh et al. reported improvement in clinical symptoms for 10 patients receiving alcohol injection, laminectomy, and instrumented fusion.³⁵ These studies suggest potential benefits from nonoperative and perioperative ethanol-based therapies, although evidence remains limited.

Lessons

Selection of appropriate AVH therapies requires consideration of lesion size, location, and symptoms, and management is often multimodal. For AVHs presenting with neurological symptoms, resection is a central component of treatment. Surgery is most frequently supplemented with embolization to minimize intraoperative blood loss, although cement augmentation may also reduce this risk. Postoperative radiotherapy can also be used to reduce recurrence risk, as seen in the 66-year-old female presented here. For select AVH cases lacking neurological symptoms, a wider range of treatments including surgery, cement augmentation, and radiotherapy can be considered as stand-alone treatments.

These findings highlight the importance of tailored AVH treatment plans guided by clinical presentation and patient treatment goals. This review is limited by its reliance on retrospective studies with small sample sizes as well as the paucity of prospective or comparative studies examining AVH management. Further research comparing long-term safety and efficacy of these modalities is warranted to better guide clinical decision-making.

Disclosures

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

Author Contributions

Conception and design: Corliss, Galgano. Acquisition of data: Benjamin, Corliss. Analysis and interpretation of data: Benjamin, Corliss. Drafting the article: Benjamin, Corliss. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Benjamin. Administrative/technical/material support: Galgano. Study supervision: Galgano.

Supplemental Information

Videos

  Video 1. https://vimeo.com/1133170758.

  Video 2. https://vimeo.com/1133827888.

Correspondence

Hughes W. Benjamin: School of Medicine, University of North Carolina at Chapel Hill, NC. hughes_benjamin@med.unc.edu.