Updated: Jun 24, 2009
Bone hemangiomas are benign, malformed vascular lesions, overall constituting less than 1% of all primary bone neoplasms. They occur most frequently in the vertebral column (30-50%) and skull (20%), whereas involvement of other sites (including the long bones, short tubular bones, and ribs) is extremely rare.
Bone hemangiomas usually occur in the medullary cavity, but uncommonly, surface-based hemangiomas are encountered in the cortex, periosteum, and subperiosteal regions.3 Gross pathology usually reveals well-demarcated, unencapsulated lesions with cystic red cavities. Microscopic examination shows hamartomatous proliferations of vascular tissue within endothelium-lined spaces.
There are 4 histologic variants of hemangioma, classified according to the predominant type of vascular channel: cavernous, capillary, arteriovenous, and venous. These types can coexist. Bone hemangiomas are predominantly of the cavernous and capillary varieties. Cavernous hemangiomas most frequently occur in the skull, whereas capillary hemangiomas predominate in the vertebral column; overall, the former type is most common in bone.4,5,6,7
Various types of nonvascular tissues may form the matrix within which the angiomatous tissue is interspersed, typically in cavernous hemangiomas. These include fat, smooth muscle, bone trabeculae, fibrous tissue, and clotted blood products. A greater proportion of fat in vertebral hemangiomas is associated with a reduced likelihood of symptoms. Conversely, neural compression is more likely to be associated with a greater proportion of hypervascular or hemangiomatous tissue.
Hemangiomas are slow growing, and malignant degeneration is virtually unknown. Rarely, locally aggressive growth patterns are recognized; hemangiomas with these patterns can mimic malignant lesions.
Epithelioid hemangiomas are benign vascular neoplasms usually occurring in the skin and superficial tissues. They are encountered uncommonly in bone. Cytologically, they may be confused with malignant tumors. Indentation or erosion of bone cortex with or without reactive bone formation may occur as a result of secondary involvement from soft-tissue hemangiomas.8
In one study, the clinical and pathologic features of 50 epithelioid hemangiomas of bone were analyzed in 29 males and 21 females 10-75 years of age (mean age, 35 years). The hemangiomas were present in long tubular bones (40%), short tubular bones of the distal lower extremity (18%), flat bones (18%), vertebrae (16%), and small bones of the hands (8%). Nine patients had involvement of more than 1 bone.8
Vertebral hemangiomas are common, with a rate of 10-12% in autopsy series. Osseous hemangiomas are less frequent at other sites.
Complications arising from osseous hemangiomas are rare, and their severity depends on the location of the lesions.
There is no documented racial variation in the frequency of hemangiomas.
Osseous hemangioma generally occurs more commonly in females than in males, with a ratio of 3:2.
The peak incidence is in the fifth decade, although osseous hemangiomas can be encountered at any age. The rare periosteal and other surface-based hemangiomas tend to occur in younger patients.
Vertebral hemangiomas are the most common benign tumor of the spinal column, and they occur most frequently in the lower thoracic and upper lumbar spine. The lesions are most often solitary, but they may be multiple in up to one third of cases. Spinal hemangiomas usually are localized to the vertebral body, less frequently extending into or exclusively affecting the posterior arch.
The large majority of lesions are asymptomatic; clinically significant symptoms develop in only 1-2% of patients. Other more common causes of back pain, such as spondylosis or disk prolapse, should be excluded before ascribing the symptoms to hemangioma. When symptoms occur, they can be vague and nonspecific. Vertebral collapse and epidural and/or extraosseous extension can result in back pain. Neural compression can produce paralysis and/or paraplegia or bladder and bowel dysfunction, whereas radicular symptoms occur from nerve-root impingement.
Calvarial lesions tend to be most significant clinically.9 Craniofacial hemangiomas may result in a palpable lump, although local pressure effects or aggressive growth patterns can cause pain. Localized swelling, limb hypertrophy, and pain can be characteristics of hemangiomas in the extremities. Hemorrhage can occur, usually in the setting of trauma, biopsy, surgery or other medical or dental interventions.
Plain radiography is useful for evaluation as the first-line imaging modality in most cases. Radiographic appearances differ depending on the anatomic site and histologic variant of the lesion. However, the radiographic hallmark of bone hemangiomas is a prominent trabecular pattern.
Radiographic patterns may be nonspecific, necessitating further imaging or histology to achieve diagnosis. This is especially true in extraspinal hemangiomas occurring in an age group and location in which other more ominous diagnostic entities, such as myeloma or metastases, are more common.
When plain radiographs do not suffice and appearances remain equivocal, cross-sectional imaging is crucial for further characterization of these lesions. CT is especially useful for assessing changes in bone trabeculae; the results support the plain radiographic findings and provide greater detail.
The superior soft-tissue and bone marrow contrast resolution of MRI allows for better evaluation of extraosseous extension and depiction of the characteristic fatty content in vertebral hemangiomas and also flow patterns in general. The multiplanar capabilities of MRI are also crucial in defining the extent of neural involvement in the spine and planning therapeutic interventions.10
Despite the added diagnostic information available with CT and MRI, the angiomatous nature of many extraspinal lesions can be confirmed only with histologic analysis.
Aneurysmal Bone Cyst
Bone Metastases
Lymphoma, Bone
Multiple Myeloma
Osteosarcoma, Classic
Paget Disease
Most vertebral hemangiomas are small and cannot be seen on plain radiographs. The characteristic radiographic appearance is of a sclerotic or ivory vertebra with coarse, thickened vertical trabeculae giving a corduroy, accordion, or honeycomb appearance (see Images 1-4). This appearance is due to resorption of horizontal trabeculae, caused by vascular channels and consequent reinforcement of vertical trabeculae. This finding can be differentiated from Paget disease, in which picture framing of the vertebral body is seen owing to prominent horizontal trabeculae. Similar findings may occur with lymphoma and metastases. Bulging of the posterior cortex or expansion of the vertebral body is sometimes present.
Calvarial hemangiomas are usually round, osteolytic lesions that may demonstrate the characteristic sunburst, radiating spoke-wheel, or weblike pattern of trabecular thickening. Radiographic appearances in craniofacial hemangiomas are often nonspecific. Mixed radiopacity, radiolucency, and honeycomb patterns are observed.
Long-bone hemangiomas are usually lytic, with a spiculated pattern creating a latticelike or Irish-lace appearance. A honeycomb structure can also result from bubbly bone osteolysis. Irregular bone destruction can occur, simulating malignant lesions. Reactive sclerosis may be seen at the margins of the lesions; with surface-based hemangiomas, they may mimic osteoid osteoma. Epithelioid hemangiomas characteristically demonstrate well-defined lysis, and they may also exhibit surrounding sclerosis, cortical expansion, or destruction.
Radiographic appearances of hemangiomas can be pathognomonic, especially with vertebral and calvarial hemangiomas. CT and MRI increase diagnostic confidence in equivocal cases.
Vertebral hemangiomas are typified by punctate sclerotic foci representing thickened vertical trabeculae seen in cross-section and giving a polka-dot appearance (see Image 5). This finding may be absent in patients with symptomatic lesions. Bulging of the posterior cortex and paravertebral soft-tissue extension are readily assessed on CT scans, as is bone destruction with aggressive hemangiomas. CT findings in nonvertebral hemangiomas confirm plain radiographic results but give more detailed assessment of medullary, cortical bone, and extraosseous involvement.
CT scanning is more sensitive than plain radiography.
MRI features largely depend on the proportion of fat and vascularity of the lesions. With T1-weighted MRI, particularly in vertebral hemangiomas, areas of high fat content appear as areas of high signal intensity. On T2-weighted images, high signal intensity typically corresponds to the vascularity of hemangiomas (see Images 6-7). Low signal intensity on T1-weighted images indicates decreased marrow fat or a greater vascular component; such a finding may be correlated with more aggressive behavior and is also more characteristic in cases involving vertebral collapse.
Thickened trabeculae demonstrate low signal intensity on MRI obtained with all sequences (see Image 8). Extraosseous components tend not to show high signal intensity on T1-weighted images owing to the paucity or absence of adipose tissue, but avid enhancement occurs with gadolinium enhancement owing to the vascularity of the lesions. Epidural extension and neural involvement are well depicted with MRI (see Images 9-10).
Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans.
NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.
Lesions in flat and long bones may show serpentine vascular channels. These demonstrate low signal intensity on T1-weighted images and high signal intensity on T2-weighted images with slow blood flow; they show low signal intensity on MRI obtained with all sequences in conditions of high blood flow.11
Complicated symptomatic spinal hemangiomas may be difficult to differentiate from malignant lesions.
Osseous hemangiomas usually show normal uptake on isotope bone scans, but they may also demonstrate photopenia and mildly to moderately increased activity. Scintigraphy with labeled red blood cells usually demonstrates focally increased activity.
Single-photon emission CT images are more sensitive than planar images in depicting abnormal activity.12
Angiographic findings confirm the hypervascularity of the lesions. Angiography usually is performed in conjunction with embolization of symptomatic hemangiomas prior to surgery.13
Hemangiomas should be treated only if symptomatic; treatment options depend on the site of the lesion, the severity of the symptoms, and the medical expertise available. Medical treatment and clinical observation can be used as first-line management, especially in patients with mild-to-moderate symptoms. Other treatment options are available when this does not suffice or when clinically appropriate.14,15
Embolization
Embolization of hemangiomas is performed prior to surgery; it helps reduce the vascularity of the lesions and, therefore, intraoperative blood loss. Embolization may also relieve cord compression by reducing lesion bulk.
Surgery
Surgery usually is reserved for refractory cases and for cases complicated by vertebral collapse with neural compression. Ideally, it is preceded by lesion embolization, and may be combined with postoperative radiation therapy, especially when pain occurs with neurologic compression. The surgical options may entail lesion excision, decompressive laminectomy, resection of epidural extension, bone grafting, and use of metallic prostheses.
Percutaneous vertebroplasty
Percutaneous vertebroplasty was introduced in France in 1984. It was first used for the treatment of vertebral hemangiomas and, subsequently, osteoporotic and malignant vertebral collapse. Vertebroplasty is minimally invasive and provides stabilization of the vertebral body and prompt, lasting pain relief that allows for early mobilization of the patients.
Vertebroplasty is ideal in the absence of cord compression or posterior arch and/or pedicle involvement, but it has also been used prior to surgical decompression to consolidate the vertebral body and reduce hemorrhagic complications.16,17 Vertebroplasty may be used in combination with embolization or ethanol injection.18
Direct ethanol injection
CT-guided direct injection of ethanol as a sclerosing agent has been used to treat vertebral hemangiomas complicated by cord and nerve root compression. This method has been shown to be effective and safe, providing symptomatic relief and lesion obliteration.
Vertebral lesions
Controversial approaches to the management of these lesions are reflected by the range of treatment options available. Options include radiation therapy, embolization, surgical resection, vertebroplasty, and intralesional injection of a sclerosant. These approaches can be used alone or in combination.
Radiation therapy is the most common treatment modality and an effective tool in the management of these lesions. It is used either as primary treatment for alleviating pain (probably as a result of its anti-inflammatory properties), for facilitating reossification, or for postsurgical care to prevent recurrence and relapse of symptoms. Obliteration of the lesion can occur with radiotherapy, but this effect is not prompt enough for treatment of cord compression.
Calvarial lesions
Curative marginal resection of these lesions can be achieved without local recurrence.
Long-bone lesions
Excision of the lesions and bone grafting are the mainstays of treatment of disease in the extremities.
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Calianeller T, Ozdemir O, Yildirim E, Kiyici H, Altinörs N. Cavernous hemangioma of temporalis muscle: report of a case and review of the literature. Turk Neurosurg. 2007;17(1):33-6. Review. [Medline].
Magliulo G, Parrotto D, Sardella B, Della Rocca C, Re M. Cavernous hemangioma of the tympanic membrane and external ear canal. Am J Otolaryngol. May-Jun 2007;28(3):180-3. Review. [Medline].
Naama O, Gazzaz M, Akhaddar A, Belhachmi A, Asri A, Elmostarchid B, et al. Cavernous hemangioma of the skull: 3 case reports. Surg Neurol. Jan 18 2008;[Epub ahead of print]:[Medline].
Madge SN, Simon S, Abidin Z, Ghabrial R, Davis G, McNab A, et al. Primary orbital intraosseous hemangioma. Ophthal Plast Reconstr Surg. Jan-Feb 2009;25(1):37-41. [Medline].
Nielsen GP, Srivastava A, Kattapuram S, Deshpande V, O'Connell JX, Mangham CD, et al. Epithelioid hemangioma of bone revisited: a study of 50 cases. Am J Surg Pathol. Feb 2009;33(2):270-7. [Medline].
Khanam H, Lipper MH, Wolf CL, Lopes MB. Calvarial haemangiomas: report of two cases and review of the literature. Surg Neurol. 2001;55(1):63-67. [Medline].
Choi JJ, Murphey MD. Angiomatous skeletal lesions. Semin Musculoskeletal Radiology. 2000;4(1):103-12. [Medline].
Ross JS, Masaryk TJ, Modic MT, et al. Vertebral haemangiomas: MR imaging. Radiology. 1987;165(1):165-9.
Han BK, Ryu JS, Moon DH, et al. Bone SPECT imaging of vertebral haemangioma correlation with MR imaging and symptoms. Clin Nucl Med. 1995;20(10):916-21.
Kahana A, Lucarelli MJ, Grayev AM, Van Buren JJ, Burkat CN, Gentry LR. Noninvasive dynamic magnetic resonance angiography with Time-Resolved Imaging of Contrast KineticS (TRICKS) in the evaluation of orbital vascular lesions. Arch Ophthalmol. Dec 2007;125(12):1635-42. [Medline].
Acosta FL Jr, Sanai N, Chi JH, Dowd CF, Chin C, Tihan T, et al. Comprehensive management of symptomatic and aggressive vertebral hemangiomas. Review. Neurosurg Clin N Am. Jan; 2008;19(1):17-29. [Medline].
Bandiera S, Gasbarrini A, De lure F, et al. Symptomatic vertebral hemangioma; the treatment of 23 cases and a review of the literature. Chir Organi Mov. 2002;87(1):1-15. [Medline].
Cortet B, Cotten A, Deprex X, et al. Value of vertebroplasty combined with surgical decompression in the treatment of aggressive spinal angioma. Apropos of 3 cases. Revue du Rhumatisme. Edition Francaise. 1994;61:16-22.
Ide C, Gangi A, Rimmelin A, et al. Vertebral Haemangiomas with spinal cord compression: the place of preoperative percutaneous vertebroplasty with methyl methacrylate. Neuroradiology. 1996;38(6):585-9.
Feydy A, Cognard C, Miaux Y, et al. Acrylic vertebroplasty in symptomatic cervical vertebral haemangiomas: report of 2 cases. Neuroradiology. 1996;38:389-91.
bone hemangioma, hemangioma, hemangioma of bone, osseous hemangioma, cavernous hemangioma, capillary hemangioma
Ishmael Chasi, MB, ChB, FRCR, Consultant Radiologist, Department of Radiology, University Hospital of North Durham, UK
Ishmael Chasi, MB, ChB, FRCR is a member of the following medical societies: Royal College of Radiologists
Disclosure: Nothing to disclose.
Geoff Hide, MBBS, MRCP, FRCR, Consultant Musculoskeletal Radiologist, Department of Radiology, Freeman Hospital; Honorary Clinical Lecturer, Faculty of Medical Sciences, University of Newcastle upon Tyne
Geoff Hide, MBBS, MRCP, FRCR is a member of the following medical societies: British Medical Association, Royal College of Physicians, and Royal College of Radiologists
Disclosure: Nothing to disclose.
Michael A Bruno, MD, Associate Professor, Departments of Radiology and Medicine, Pennsylvania State University College of Medicine; Director, Radiology Quality Management Services, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine
Michael A Bruno, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, Society of Nuclear Medicine, and Society of Skeletal Radiology
Disclosure: Nothing to disclose.
Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.
Theodore E Keats, MD, Professor, Departments of Radiology and Orthopedics, University of Virginia School of Medicine
Disclosure: Nothing to disclose.
Robert M Krasny, MD, Consulting Staff, Department of Radiology, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.
Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.
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