Vertebral Hemangioma – Causes, Symptoms, Treatment

Vertebral Hemangioma (VH) is a virtually vascular malformation, which is usually asymptomatic. Only 3.7 % of VH may become active and symptomatic, and 1 % may invade the spinal canal and/or paravertebral space. Treatment protocols for active or aggressive VHS are still in controversy. Reported treatments include radiotherapy, vertebroplasty, direct alcohol injection, embolization, surgery, and a combination of these modalities.

Vertebral body hemangioma is a common condition with various case series reporting its incidence to be between 10% and 27% [rx]. In the bone, they are of dysembryogenetic origin otherwise also called as hamartomatous lesions. They have high vascularity, intralesional edema, and intralesional fat [rx]. Most hemangiomas are small, about a third are multiple and only 0.9-1.2% of hemangiomas cause symptoms [rx, rx]. Very few hemangiomas are aggressive causing bony expansion and extraosseous extension, with extension into the paraspinal and epidural spaces [rx, rx, rx]. New onset of back pain with the subacute progression of thoracic myelopathy has been reported as the most common presentation for patients with neurological deficits due to aggressive vertebral body hemangiomas [rx]. Thus, it is important to recognize this entity, include it in the clinical differential diagnosis and to look for the extensions of such vertebral body hemangiomas.

Vertebral hemangiomas are most commonly restricted to the vertebral body.[rx] Infrequently, examples of aggressive hemangiomas extending to the posterior elements have been described, and these more frequently cause spinal cord compression. Very few cases of hemangiomas restricted to the posterior elements have been reported in the literature. Here, we present a rare case of hemangioma restricted to the posterior spinal elements causing spinal cord compression. The embolization of this lesion preoperatively was not possible due to the relation of the tumor to a prominent radiculomedullary artery.

Causes of Vertebral Hemangioma

The vast majority of these are asymptomatic. Infrequently, these can turn symptomatic and cause neurological deficit (cord compression) through any of four reported mechanisms:

• (1) epidural extension;
• (2) expansion of the involved vertebra
• (e) causing spinal canal stenosis;
• (3) spontaneous epidural hemorrhage;
• (4) pathological burst fracture. Thoracic haemangiomas have been reported to be more likely to produce cord compression than lumbar haemangiomas.

Symptoms of Vertebral Hemangioma

Most hemangiomas are asymptomatic. The collapse of the vertebral body or encroachment into the neural canal are some of the classic causes of pain. An increase in activity can cause the vertebral hemangioma to become painful, such as starting to exercise, housework and such. This is most likely due to axial loading through the body of the vertebra.

Symptoms vary depending on the size and location of the tumor; most hemangiomas cause no symptoms at all.

Among symptomatic hemangiomas, the most common symptom is pain at the tumor site, usually in the back. If the tumor expands beyond the bony boundaries or causes collapse of a vertebra it will create pressure on the surrounding nerves or spinal cord. This can lead to pain that radiates to the arms or legs, weakness, numbness, or clumsiness of the arms or legs, or compromised bowel and/or bladder control.

A vertebra weakened by a hemangioma may collapse, a painful event known as a compression fracture. Hemangiomas may also bleed, causing a hemorrhage that can expand and compress the nerves or spinal cord.

Diagnosis of Vertebral Hemangioma

Physical examination

• Physical examination primarily involves a thorough back examination that includes a visual inspection of the overlying skin, assessing the curvature of the spine, and observing gait. The range of motion should also be tested including forward flexion, extension, lateral flexion, and rotation.
• The spine should be palpated specifically at the patient-indicated site of pain. Percussion should be performed to assess for costovertebral tenderness. Considering hemangiomas can erode into the spinal canal, assessing for radiculopathy is important in the physical examination of the spine.
• Special maneuvers to asses for radiculopathy should be performed including the straight leg raise test for the lower back and the Spurling maneuver in the setting of cervical radiculopathy. Reflexes should be tested, and sensation with close attention to the thoracic dermatomes should be performed. Finally, the physical examination should include evaluation of the gastrointestinal and genitourinary systems.

Treatment of Vertebral Hemangioma

In general, treatment for vertebral hemangiomas should entirely base on patients’ symptoms, for even the aggressive VH lesions are benign in nature and rarely progress rapidly.

Symptomatic VHs have been treated with surgery, transarterial embolization, direct ethanol injection, radiotherapy, and vertebroplasty, each with varying degrees of success.^[rx] Each of these methods can be indicated in specific clinical settings.

• For type I VHs – only observation is needed [rx].
• In contrast to type I VHs – treatment protocols for active or aggressive VHS are still in controversy. For type II VHS, radiotherapy was used to be the first of choice. Its vascular necrosis and/or anti-inflammatory effect acts to obliterate hemangiomas and control pain. The effective dosage is 30–40 Gy over a 3- to 4-week period and the associated complications, such as radionecrosis, radiation-induced myelitis, and secondary malignancy are rare. Nevertheless, the fear for its secondary malignancy had prompted the development of new treatment, i.e., vertebroplasty. More recently, however, Heyd et al. [rx] point out that secondary malignancy has been over-emphasized, and vertebroplasty has its own fatal complications such as leakage and pulmonary embolism. In most VH cases, the vertebral trabecular tend to be thicker and stronger than the normal vertebral trabecular, hence no vertebral augmentation is needed. Vertebroplasty could cure VH by obstructing the vessels of tumors (internal embolization).
• As for type III VHs – the importance of early diagnosis and treatment to prevent neurological deficit has been emphasized by Doppman and his colleagues [rx]. However, it is argued by Boriani and colleagues who insist that VH is pathologically benign and treatment should be symptoms orientated [rx]. To them, this type of indolent VH lesions is not really aggressive even with extra compartment extension hence preventive treatments are not mandatory. For us, we prefer observation, but spinal radiosurgery is also a reasonable choice. We inform the patient and his/her family, and they make the treatment decision after a comprehensive consultation.
• Treatment protocol for type IV VHS – is even more diversiform. Reported treatments include radiotherapy [rx], vertebroplasty, direct alcohol injection [rx], embolization of the feeding arteries, surgery, and a combination of these modalities. Successful results have been reported for each of these treatments. But the corresponding technical requirement, learning curve, complications, and costs vary greatly. Besides, it is difficult to assess these treatments individually as most reports were retrospective case reports with mixed cases with local pain alone and most patients had combined treatments.
• Radiotherapy – alone is only appropriate for patients with slight and slow progressive neurological deficits because of the concern that the effect of radiotherapy is not as prompt as desired for patients with progressive neurological deficits caused by cord compression. Spinal radiosurgical treatment could lead to better neurological outcomes by focusing on epidural lesion [rx]. Surgery is indicated if radiotherapy fails in 3 months’ time. Heyd and colleagues reported that neurological symptoms completely resolved in 79 % of their cases after radiotherapy [rx]. But amongst the 24 cases with neurological symptoms in their retrospective multicenter study, 20 also had surgical intervention.
• Vertebroplasty or kyphoplasty – Jankowski et al. (19) considered patients with pathologic vertebral fractures and persistent pain eligible for percutaneous vertebroplasty or kyphoplasty but they stated that vertebroplasty is a contraindication if VH expands into the spinal canal. In kyphoplasty, in fact, the risk of major complications as the cement leakage is present. Fourney et al. (20) proposed the intraoperative vertebroplasty following laminectomy to achieve direct visualization during the procedure to reduce this risk.
• Vertebroplasty – is not suggested for type IV lesions, for it might exacerbate cord compression. Instead, it is safe to carry out intraoperative vertebroplasty after laminectomy under direct visualization. In some aggressive cases where both trabecular and the cortex are destructed, vertebroplasty can strengthen the vertebral bodies and prevent vertebral collapse. After radiotherapy, the radiological ossification rate was only 26 % [rx].
• Direct alcohol injection – into the tumor is highly effective in achieving hemostasis. It causes intralesional thrombosis and destruction of the endothelium of which the hemangioma composed. Devascularisation is followed by shrinkage of the lesion, which decompresses the cord and nerve roots. CT angiography revealed that these hypervascular components are often inhomogeneous [rx], and the injection targeted the most hypervascular subsection. A high success rate was reported by Heiss, Losner, and Doppman [rx]. More recently in India, the direct injection has been carried out during decompressive surgery in VH cases. The complications include transient neurological deterioration (including Brown–Sequard syndrome), pathologic fractures, and recurrence. Doppman et al. [rx] suggested that a moderate dose of ethanol (<15 ml) obliterated the hemangioma without affecting spinal stability. In Bas’ series, 6 out of 24 patients were not ideal for alcohol injection because the contrast medium was not retained in the lesions [rx].
• The embolization – of feeding vessels has been used widely as a presurgical adjuvant treatment for reducing intraoperative hemorrhage. Kawahara and colleagues had indicated that the interruption of bilateral segmental arteries at three levels does not damage spinal cord function in either dog or human [rx]. But it may not be possible in the absence of a discernible feeding vessel. With the development of intraoperative vertebroplasty and/or direct alcohol injection, preoperative embolization for VH might not be needed anymore.

Surgery

• Operation – is indicated in cases with rapidly progressive and/or severe myelopathy. Sometimes, urgent surgical decompression is indicated. However, diagnostic failure can potentially lead to inappropriate surgical plan and serious complications, especially life-threatening hemorrhage. Before 1960, the reported surgical neurological recovery rate was on average 73 % (range 43–85 %), and the mortality rate was 11.7 % [rx]. In recent studies, however, the neurological recovery rate is nearly 100 %, whereas the mortality is rarely mentioned [rx]. The goals of surgery include bony decompression and excision of soft tissue components of the tumor that compress the neural elements. Different surgical techniques have been reported, namely, laminectomy [rx], corpectomy, spondylectomy [rx], even total en-bloc spondylectomy (TES) [rx, rx].
• Laminectomy – could be combined with intraoperative vertebroplasty or alcohol injection, which could shrink the hemangiomas and lead to intralesional thrombosis. In most type IV cases, it is the epidural soft tumor mass in stead of the bony lesion which causes neurological deficit. Appropriate decompression could be achieved without anterior decompression. After laminectomy, alcohol or cement leakage could be easily controlled under direct visualization. With these techniques, preoperative embolization might no longer be needed. Although postoperative radiotherapy plays an important role in reducing reoccurrence in cases of partial tumor excision [rx], it may not be needed in cases with ideal intraoperative vertebroplasty or alcohol injection.
• Spondylectomy – is too radical even for aggressive VH, considering its benign nature. It may be specifically indicated for ventrally located lesions with bony compression. Given the VHs are usually highly vascularised, it can cause substantially great intraoperative blood loss. Even with the improvement of surgical technique, it remains an extremely technically demanding procedure. With preoperative embolization, the average blood loss was 2.1 L (range 0.8–5 L) in the 10 spondylectomy cases reported by Acosta et al. [rx]. The estimated blood loss was 4.0 L in the TES case reported by Inoue et al. [rx].

References

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