Vertebral hemangiomas: A report of two unusual cases and a review of literature

Suresh S Pillai; M Harisankar

doi:10.4103/joasis.joasis_8_20

CASE REPORT

Year : 2020 | Volume : 17 | Issue : 1 | Page : 28-33

Vertebral hemangiomas: A report of two unusual cases and a review of literature

Suresh S Pillai¹, M Harisankar²
¹ Department of Spine Surgery, Baby Memorial Hospital, Kozhikode, Kerala, India
² Department of Orthopedics, Baby Memorial Hospital, Kozhikode, Kerala, India

Date of Submission	22-Oct-2020
Date of Decision	10-Nov-2020
Date of Acceptance	27-Oct-2020
Date of Web Publication	17-Nov-2020

Correspondence Address:
M Harisankar
Sarika, Perunnai West PO, Changanacherry 2, Kottayam - 686 102, Kerala
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joasis.joasis_8_20

Abstract

Vertebral hemangiomas are among the most common benign lesions of the spine, with an incidence of 10%–12% in the adult population. Vertebral hemangiomas have been reported in all age groups with most prevalence in the fifth decade of life. A small proportion of the lesions (1%) may cause neurological symptoms ranging from pain to full-fledged paraplegia. Since most of the vertebral hemangiomas are asymptomatic and quiescent, no treatment is required. Treatment is to be considered only if there is back pain or neurological symptoms due to vertebral fracture or spinal cord/root compression. Because these active and symptomatic types of vertebral hemangiomas are a rare occurrence, their treatment modalities remain controversial and hence problematic. Since most of the patients are treated by a combination of modalities, the analysis of the efficacy of each treatment is difficult to assess and hence the apt choice of treatment is still controversial.

Keywords: Hemangioma, spine tumor, treatment of hemangioma

How to cite this article:
Pillai SS, Harisankar M. Vertebral hemangiomas: A report of two unusual cases and a review of literature. J Orthop Assoc South Indian States 2020;17:28-33

How to cite this URL:
Pillai SS, Harisankar M. Vertebral hemangiomas: A report of two unusual cases and a review of literature. J Orthop Assoc South Indian States [serial online] 2020 [cited 2023 Apr 1];17:28-33. Available from: https://www.joasis.org/text.asp?2020/17/1/28/300761

Introduction

Vertebral hemangiomas are among the most common benign lesions of the spine, with an incidence of 10%–12% in the adult population.^[1],[2] They are usually described as dysplasias or vascular malformations affecting the vertebral column and are attributed to dysembryogenetic disturbances affecting the proper differentiation of blood vessels.^[3],[4] Majority of the vertebral hemangiomas are quiescent and asymptomatic. A small proportion of the lesions (1%) may cause neurological symptoms ranging from pain to full-fledged paraplegia.^[5] Because these active and symptomatic types of vertebral hemangiomas are a rare occurrence, their treatment modalities remain controversial and hence problematic. The main objective of this article is to summarize the natural history, radiological findings, pathology, and treatment options of vertebral hemangiomas.

Case Reports

Case 1

A 36-year-old female with a history of low back pain was initially treated by ayurvedic massage. Following the massage, she developed weakness of both lower limbs which soon progressed to dense paraplegia. She was taken to a hospital nearby. On magnetic resonance imaging (MRI) evaluation, she was diagnosed to have an aggressive hemangioma of D12 and L1 causing cord compression [Figure 1]a and [Figure 1]b. She was prepared for decompression and stabilization. However, intraoperatively, she developed torrential and uncontrollable bleeding causing hypotension. The wound was packed since the bleeding could not be controlled and was referred to our institution. On arrival, the patient was managed with blood transfusion and fluids. An interventional radiology consultation and embolization of the feeding vessels to the tumor was done [Figure 2]. After the embolization, we could attain hemostasis, successful decompression of the cord, and posterior stabilization of the spine [Figure 3]. She was not given any postoperative radiotherapy. The patient had a gradual full motor and sensory recovery by the end of 6 months. She has been on follow-up for the past 15 years and is doing well without any further incidents.

Figure 1: (a and b) Magnetic resonance imaging showing hemangioma of D12 and L1 causing cord compression

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Figure 2: Angioembolization of the lesion

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Figure 3: Intraoperative picture showing decompressed cord and posterior stabilization

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Case 2

A 40-year-old male presented to the outpatient department (OPD) with back pain and gait disturbances. On detailed MRI evaluation, he was diagnosed to have a hemangioma of D12 vertebra causing cord compression [Figure 4]a, [Figure 4]b, [Figure 4]c. He was subjected to preoperative angioembolization of the feeding vessels of the lesion [Figure 5], and after 24 h, he was treated with a 360° surgery with decompression, corpectomy of D12 vertebra, and iliac crest strut graft placement through the anterior approach [Figure 6] and posterior stabilization through the posterior approach [Figure 7] and [Figure 8]a and [Figure 8]b. The tissue was sent for histopathological evaluation which revealed hemangiomatous tissue along with the presence of an increased number of plasma cells. The patient recovered his functions within 3 months of the surgery and returned to his routine activities by the end of 6 months.

Figure 4: (a-c) Magnetic resonance imaging showing a hemangioma of D12 vertebra causing cord compression

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Figure 5: Preoperative angioembolization of the feeding vessels of the lesion

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Figure 6: Intraoperative picture showing corpectomy and anterior decompression

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Figure 7: Posterior stabilization – intraoperative picture

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Figure 8: (a and b) Postoperative radiographs

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After 7 years, the patient presented again with back pain, and now, the MRI evaluation showed the D9 and D10 vertebral bodies affected with a lytic lesion. A biopsy taken from the tumor revealed it to be multiple myeloma. The patient was referred to the oncology department for further medical management of the tumor. One year later, the patient presented to the OPD with back pain and a clicking sensation at the surgical site. Radiographic evaluation revealed a broken rod. He was treated with a replacement of the broken rod. After another year, he came to visit again with a similar clicking sensation and pain, and it was found that the rod on the opposite side was broken. He was treated with the replacement of the rod. He is continuing his medical oncological treatment and follow-up. He has been on follow-up for the past 2 years and with no new symptoms.

Discussion

Clinical features

Vertebral hemangiomas have been reported in all age groups with most prevalence in the fifth decade of life. This lesion has a female preponderance, with a male: female ratio of 1:1.5.^[1],[6],[7] The most common location is the thoracic spine, though occurrences have been noted in the cervical as well as lumbar spine. Mostly, they involve the vertebral body but rarely may involve the posterior arch as well.^[1],[8]

Usually, the vertebral hemangiomas are asymptomatic and are incidental findings, but rarely, the lesions can be active and cause compression of the spinal cord and cause neurological symptoms. These varieties are called compressive hemangiomas.^[7] The symptoms range from back pain, radiculopathies to myelopathy and even paraplegia. The compression can be due to bone expansion, expansion of the lesion into the spinal canal, vascular occlusion of the spinal cord, epidural hemorrhage, or even mechanical compression due to the collapse of the affected vertebra.^[9],[10] The most common location of the compressive type of vertebral hemangioma is the thoracic spine and is mostly found in younger individuals.

Vertebral hemangioma and pregnancy

Women with vertebral hemangiomas usually become symptomatic in the third trimester of pregnancy owing to the increased intra-abdominal pressure leading to increased blood flow within the vertebral venous system or due to relatively sparse vascular supply of the thoracic spinal cord.^[3],[11] In addition, the endothelial growth-promoting effect of the elevated estrogen levels results in the increased size of the hemangioma during pregnancy.

Histopathological features

Characteristically, these are benign tumors composed of abnormal blood vessels that may be capillary sized or cavernous in nature.^[12] Cavernous type is the most common, with large sinusoidal spaces covered with a single layer of epithelium. Macroscopically, the tissue is soft in consistency, well demarcated, and often dark red in color. The bony trabeculae of the involved vertebral body are thickened and sclerotic imparting it a honeycomb-like appearance. Fibrous/adipose involution of bone marrow is also observed frequently.^[7],[12]

Radiological features

Based on radiological features, vertebral hemangiomas can be classified as typical, atypical, and aggressive.^[13] The classification into typical and atypical is based on the ratio of fatty to vascular components in the tissue and interstitial edema which becomes apparent on MRI evaluation. The aggressive group is identified by the extension of the lesion beyond the vertebral body, destruction of the vertebral cortex, and the invasion of epidural and paravertebral spaces.^[7],[9],[13]

The lesions with a high fatty content are usually benign or quiescent, whereas lesions with a higher vascular content have the chance to evolve into an atypical variant and may even become aggressive and cause compressive symptoms.^[9]

Paravertebral soft-tissue involvement that is seen in conjunction with vertebral hemangiomas may represent hematoma or extravertebral tumor extension. This can be seen anterior or posterior to the vertebral body.^{[3],[14],[15]}

Typical vertebral hemangiomas

The characteristic radiographic features include reduced bone density between much denser and sclerotic vertebral trabeculae in a nonexpanded vertebral body. This gives the vertebral hemangiomas a typical honeycombed or jail-bar appearance commonly referred to as the corduroy pattern.^[16] This vertical jail-bar pattern is typically seen in a lateral view due to the resorption of the horizontal trabeculae and consequent reinforcement of the vertical trabeculae.^[7],[9] It should be noted that at least one-third of the vertebral body should be involved for the radiographic signs to be apparent.^[17] The disc spaces are usually intact.^[15]

Computed tomography (CT) imaging shows the typical polka-dot sign which is pathognomonic for vertebral hemangioma.^[18] MRI shows a characteristic appearance due to the serpentine vascular channels in the hemangiomatous lesion. The higher fat content in the lesion makes it hyperintense in T1-weighted images, and the higher vascular content makes it hyperintense in the fluid-sensitive T2-weighted images.^[16]

Atypical vertebral hemangiomas

Atypical vertebral hemangiomas have a higher vascular to fatty content ratio compared to the typical vertebral hemangiomas. Hence, in MRI, the lesion might appear hypointense in T1-weighted images. However, the lesion will be hyperintense in the T2-weighted images due to the higher vascular content.^[9]

Aggressive vertebral hemangiomas

These are different from the usual vertebral hemangiomatous lesions in that the characteristic radiographic findings may be absent. The nonspecific findings that are commonly seen with such lesions are expanded cortex, osteoporosis, collapse of the vertebral body, erosion of the pedicles, and the presence of lytic areas.^[7],[9],[13]

Laredo et al.^[9] proposed radiographic criteria for the identification of aggressive vertebral hemangiomas. They described six radiographic and CT features and proposed that if more than three are present along with radicular pain, that indicates that the lesion is of the aggressive variety. The features that were described were as follows:

Involvement of the entire vertebral body
Extension into the neural arch
Cortical expansion
Thoracic location (T3–T9)
Irregular honeycomb pattern
Soft-tissue mass.

Other radiographic features that may point toward an aggressive lesion are a maintained vertebral body height, a sharp margin with normal marrow, intact cortex adjacent to a paraspinal mass, and enlarged paraspinal vessels.^[10] Before the treatment of aggressive vertebral hemangiomas, an angiography of the lesion to identify the feeding/draining vessels is recommended. The feeding vessels are usually a branch of a lumbar or intercostal artery that arises proximal to the radicular branches.^[19] The typical angiography findings include dilatation of the arterioles, numerous blood pools in the capillary phase, and intense opacification extending beyond the normal hemivertebral territory throughout the entire vertebral body.^[9]

Differential diagnosis

Conditions such as focal fatty replacement, Modic type II changes, and prior radiation therapy lead to lesions that look like typical hemangiomas on radiographic evaluation. The MRI findings of atypical vertebral hemangiomas are similar to those of metastatic disease and even plasmacytoma.^[7] The lesions that share a similar radiographic picture with an aggressive variety of vertebral hemangiomas include solitary bone plasmacytoma, chordoma, lymphoma, and epithelioid hemangioendothelioma.^[16],[20] Aggressive vertebral hemangiomas present a diagnostic challenge to the radiologist since there is no radiologic finding characteristic to it. Vertebral hemangiomas may coexist with other lesions, further making the diagnosis difficult. In such cases, angiography becomes an essential and useful diagnostic tool. A biopsy may be necessary if the diagnosis is not confirmed with imaging alone.^[7]

Another differential is cystic angiomatosis of bone which is associated with widespread cystic bone destruction, visceral angiomas, and generally a poor prognosis.^[21],[22]

Plasmacytomas and hemangiomas are common findings in patients, but a coexisting hemangioma and plasmacytoma in a single vertebra is an extremely rare scenario with only two such cases reported previously in the literature.^[23]

Treatment

Since most of the vertebral hemangiomas are asymptomatic and quiescent, no treatment is required. Treatment is to be considered only if there is back pain or neurological symptoms due to vertebral fracture or spinal cord/root compression.^[24] The treatment options available for symptomatic vertebral hemangiomas are conservative therapy with medications, percutaneous techniques (including but not limited to vertebroplasty, transarterial embolization, and ethanol injection), radiotherapy, and surgery. Since hemangiomas rarely require treatment, and most of the patients are treated by a combination of the abovementioned modalities, the analysis of the efficacy of each treatment is difficult to assess and hence the apt choice of treatment is still controversial.

Surgery: Urgent surgical decompression is indicated only in patients with compressive hemangiomas causing neurological symptoms. Surgical treatment involves excision (corpectomy/spondylectomy) or decompression or both.^[25] Excision is associated with significant blood loss and hence preoperative embolization of the feeding vessels may be considered. Compared to excision, decompression is associated with limited blood loss.^[5] Stabilization of the spine should be considered if the resection of the tumor tissue compromises the intrinsic stability of the spine. Anterior strut grafting is considered if corpectomy is done. Posterior stabilization is preferred if the surgery that is performed is wide laminectomy and resection^[3]
Vertebroplasty: The injection of methyl methacrylate into the vertebra increases the strength of the vertebral body and thus prevents the vertebral body collapse in case of severe bony destruction.^[26] It has also been reported that the methyl methacrylate injection causes intralesional thrombosis, thus causing healing of the lesion. In cases with compressive symptoms, vertebroplasty can be performed after decompressing the cord through laminectomy
Ethanol injection: Direct ethanol injection into the lesion causes intralesional thrombosis and destruction of the endothelium of the hemangioma. This eventually causes shrinkage of the lesion, thus relieving the compressive symptoms^[5]
Radiotherapy: Vertebral hemangiomas are radiosensitive lesions affected by the administration of 3000–4000 cGy.^[27],[28] It can control the lesion by vascular necrosis and anti-inflammatory effect. Radiotherapy is ineffective as a sole mode of therapy but is used as an adjuvant to surgery to prevent recurrence of the lesion and to eliminate the leftover tumor cells after incomplete surgical removal.^[5],[12] In fact, subtotal resection of tumor tissue followed by radiotherapy has been the preferred mode of treatment for these lesions^[3]
Percutaneous transarterial embolization: Hekster et al. reported reversal of spinal cord compression following percutaneous embolization of the feeding vessels.^[29] This mode of treatment can hence be used as the sole mode of therapy though most of the surgeons prefer to use this as a presurgical adjunct to reduce the blood loss during resection/decompression surgeries.^[5]

Prognosis

Fat predominant lesions are benign in nature when compared to lesions with increased vascular content which have a higher propensity for turning aggressive. Hence, lesions with increased fat content provide a better prognosis. Malignant degeneration of hemangiomas is not known to occur.^[3],[27]

Conclusion

Although vertebral hemangiomas are very common lesions of the spine that is encountered in clinical practice, most of them are asymptomatic and hence do not require any treatment. The atypical and aggressive types of hemangiomas that require treatment need to be identified by clinical and radiological evaluation. The classical treatment includes decompression/excision of the lesion with stabilization of the spine, followed by radiotherapy for residual lesion. The use of preoperative angioembolization of the feeding vessels to the tumor is a very crucial step, since not doing that may result in torrential bleeding at the time of surgery which can lead to hypotension and even shock. Proper histopathological evaluation of the tumor tissue is warranted to rule out any coexisting plasmacytoma. Proper close follow-up of these patients is of paramount importance as some of these lesions may recur after some time. If hemangiomas are not promptly diagnosed and managed with the utmost care, the patient can develop disastrous neurological symptoms and even paraplegia.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Rodallec MH, Feydy A, Larousserie F, Anract P, Campagna R, Babinet A, et al. Diagnostic imaging of solitary tumors of the spine: What to do and say. Radiographics 2008;28:1019-41.
2.	Huvos AG. Hemangioma, Lymphangioma, Angiomatosis/Lymphangiomatosis, Glomus Tumor. Bone Tumors: Diagnosis, Treatment, and Prognosis. 2^nd ed.. Philadelphia: Saunders; 1991. p. 553-78.
3.	Fox MW, Onofrio BM. The natural history and management of symptomatic and asymptomatic vertebral hemangiomas. J Neurosurg 1993;78:36-45.
4.	Slon V, Stein D, Cohen H, Sella-Tunis T, May H, Hershkovitz I. Vertebral hemangiomas: Their demographical characteristics, location along the spine and position within the vertebral body. Eur Spine J 2015;24:2189-95.
5.	Jiang L, Liu XG, Yuan HS, Yang SM, Li J, Wei F, et al. Diagnosis and treatment of vertebral hemangiomas with neurologic deficit: A report of 29 cases and literature review. Spine J 2014;14:944-54.
6.	Campanacci M. Bone and soft tissue tumors: clinical features, imaging, pathology and treatment. Springer Science & Business Media; 2013 Jun 29.
7.	Gaudino S, Martucci M, Colantonio R, Lozupone E, Visconti E, Leone A, et al. A systematic approach to vertebral hemangioma. Skeletal Radiol 2015;44:25-36.
8.	Baudrez V, Galant C, Lecouvet FE, Malghem J, Maldague BE, Vande Berg BC. Vertebral hemangioma: MR-histological correlation in autopsy specimens. In: Radiology. Easton, PA 18042 USA: Radiological Society North America 20^th and Northampton STS; 1999. p. 245.
9.	Laredo JD, Assouline E, Gelbert F, Wybier M, Merland JJ, Tubiana JM. Vertebral hemangiomas: Fat content as a sign of aggressiveness. Radiology 1990;177:467-72.
10.	Friedman DP. Symptomatic vertebral hemangiomas: MR findings. AJR Am J Roentgenol 1996;167:359-64.
11.	Chi JH, Manley GT, Chou D. Pregnancy-related vertebral hemangioma. Case report, review of the literature, and management algorithm. Neurosurg Focus 2005;19:E7.
12.	Hart JL, Edgar MA, Gardner JM. Vascular tumors of bone. InSeminars in Diagnostic Pathology 2014;31:30-38. WB Saunders.
13.	Cross JJ, Antoun NM, Laing RJ, Xuereb J. Imaging of compressive vertebral haemangiomas. Eur Radiol 2000;10:997-1002.
14.	Baker ND, Klein MJ, Greenspan A, Neuwirth M. Symptomatic vertebral hemangiomas: A report of four cases. Skeletal Radiol 1986;15:458-63.
15.	Mohan V, Gupta SK, Tuli SM, Sanyal B. Symptomatic vertebral haemangiomas. Clin Radiol 1980;31:575-9.
16.	Ross JS, Masaryk TJ, Modic MT, Carter JR, Mapstone T, Dengel FH. Vertebral hemangiomas: MR imaging. Radiology 1987;165:165-9.
17.	Healy M, Herz DA, Pearl L. Spinal hemangiomas. Neurosurgery 1983;13:689-91.
18.	Persaud T. The polka-dot sign. Radiology 2008;246:980-1.
19.	Hemmy DC. Vertebral hemangiomas. Neurosurgery. 1996:1827-9.
20.	Alexander J, Meir A, Vrodos N, Yau YH. Vertebral hemangioma: An important differential in the evaluation of locally aggressive spinal lesions. Spine (Phila Pa 1976) 2010;35:E917-20.
21.	Zito G, Kadis GN. Multiple vertebral hemangiomas resembling metastases with spinal cord compression. Arch Neurol 1980;37:247-8.
22.	Boyle WJ. Cystic angiomatosis of bone. A report of three cases and review of the literature. J Bone Joint Surg Br 1972;54:626-36.
23.	Melcher C, Wegener B, Niederhagen M, Jansson V, Birkenmaier C. An intramedullary capillary hemangioma of the spine with an underlying plasmocytoma. Spine J 2013;13:e1-4.
24.	Dang L, Liu C, Yang SM, Jiang L, Liu ZJ, Liu XG, et al. Aggressive vertebral hemangioma of the thoracic spine without typical radiological appearance. Eur Spine J 2012;21:1994-9.
25.	Tomita K, Kawahara N, Murakami H, Demura S. Total en bloc spondylectomy for spinal tumors: improvement of the technique and its associated basic background. J Orthop Sci 2006;11:3-12.
26.	Guarnieri G, Ambrosanio G, Vassallo P, Pezzullo MG, Galasso R, Lavanga A, et al. Vertebroplasty as treatment of aggressive and symptomatic vertebral hemangiomas: Up to 4 years of follow-up. Neuroradiology 2009;51:471-6.
27.	Faria SL, Schlupp WR, Chiminazzo H Jr. Radiotherapy in the treatment of vertebral hemangiomas. Int J Radiat Oncol Biol Phys 1985;11:387-90.
28.	Glanzmann C, Rust M, Horst W. Irradiation therapy of vertebral angionomas: Results in 62 patients during the years 1939 to 1975 (author's transl). Strahlentherapie 1977;153:522-5.
29.	Hekster RE, Luyendijk W, Tan TI. Spinal-cord compression caused by vertebral haemangioma relieved by percutaneous catheter embolisation. Neuroradiology 1972;3:160-4.