Review Article

Benign Tumors of the Spine

Abstract
Nikhil A. Thakur, MD Alan H. Daniels, MD Jonathan Schiller, MD Mauricio A. Valdes, MD John K. Czerwein, MD Alan Schiller, MD Sean Esmende, MD Richard M. Terek, MD

Benign tumors in the spine include osteoid osteoma, osteoblastoma, aneurysmal bone cyst, osteochondroma, neurobroma, giant cell tumor of bone, eosinophilic granuloma, and hemangioma. Although some are incidental ndings, some cause local pain, radicular symptoms, neurologic compromise, spinal instability, and deformity. The evaluation of spinal tumors includes a thorough history and physical examination, imaging, sometimes laboratory evaluation, and biopsy when indicated. Appropriate treatment may be observational (eg, eosinophilic granuloma) or ablative (eg, osteoid osteoma, neurobroma, hemangioma), but generally is surgical, depending on the level of pain, instability, neurologic compromise, and natural history of the lesion. Knowledge of the epidemiology, common presentation, imaging, and treatment of benign bone tumors is essential for successful management of these lesions.

From the Department of Orthopaedics, State University of New York Upstate Medical University, Syracuse, NY (Dr. Thakur), The Warren Alpert Medical School of Brown University, Providence, RI (Dr. Daniels), the Department of Orthopaedics, Rhode Island Hospital/Brown University, Providence (Dr. J. Schiller, Dr. Esmende, and Dr. Terek), the Department of Orthopaedic Surgery, University of Arizona, Tuscon, AZ (Dr. Valdes), the Center for Orthopaedics, Johnston, RI (Dr. Czerwein), and the Department of Pathology, Mt. Sinai School of Medicine, New York, NY (Dr. A. Schiller). J Am Acad Orthop Surg 2012;20: 715-724 http://dx.doi.org/10.5435/ JAAOS-20-11-715 Copyright 2012 by the American Academy of Orthopaedic Surgeons.

rimary benign spine tumors are rare. Approximately 1% of all primary skeletal tumors are benign spine tumors.1 They may be incidental findings, or they may cause local pain, radicular symptoms, neurologic compromise, spinal instability, and deformity. Clinical evaluation of spinal tumors includes a thorough history and physical examination, imaging, sometimes laboratory evaluation, and biopsy when indicated, particularly when malignancy is a possibility. Appropriate management of benign tumors may be observational, ablative, or surgical depending on level of pain, instability, neurologic compromise, and natural history of the lesion. Primary benign spine tumors are classified using the Enneking system (Table 1), and generally stage II and III lesions require treatment. A thorough knowledge of the epidemiology, common presentation, imaging, and treatment of benign bone tumors is essential for successful management of these lesions.

Osteoid Osteoma
Osteoid osteomas are bone-forming tumors that are small in size (15 to 20 mm), have limited growth potential, cause pain, and account for approximately 10% of primary bone tumors.2,3 More than 50% of osteoid osteomas occur in the long bones of the lower extremity, and approximately 10% occur in the vertebrae, primarily in the posterior elements.2

Presentation
Patients most commonly present with back pain, which typically begins as mild and intermittent but can become more severe and constant over time. Symptoms can persist for weeks to years before presentation and often occur at night.2 Anti-inflammatory medications such as salicylates, nonsteroidal anti-inflammatory drugs, and cyclooxygenase-2 inhibitors characteristically provide pain relief.4 The average age of presentation is

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Table 1 Enneking Classication of Benign Spine Tumors Stage I II Description Latent, usually asymptomatic, discovered incidentally Active lesions, present with symptoms (pain in area of the lesions) Locally aggressive with possible metastases

Figure 1

III

Axial CT scan of an osteoid osteoma (arrow). (Courtesy of Imad Azouka, MD, Joslin Diabetes Center, Bahrain, 2012.)

19 years, with >80% of patients presenting before age 30 years.2 Males are affected more commonly than females. Osteoid osteoma is the most common cause of painful scoliosis in the adolescent population.5 The deformity is thought to be secondary to muscle spasm and is initially flexible but can become rigid if left untreated. The osteoid osteoma lesion typically occurs on the concavity of the curve. Neurologic symptoms are not typically observed.

localize an osteoid osteoma as an area of intense uptake.7 Osteoid osteomas are composed of woven bone (nidus), seen as interconnecting trabeculae, sheets, or individual islands, with a rim of plump osteoblasts. The tissue between the woven bone is composed of loose fibrovascular tissue with prominent osteoclasts. The bone surrounding the lesion may be composed of both lamellar and woven bone.

Evaluation and Pathology

Laboratory studies in patients with osteoid osteoma are typically within normal limits. Radiographic evaluation may not initially reveal the lesion, or it may reveal only a subtle area of sclerosis. Lesions typically consist of sclerosis surrounding a radiolucent nidus, which is by classic definition 15 to 20 mm in diameter.2,3 Osteoid osteoma is more clearly seen with CT6 (Figure 1). MRI is effective in determining their proximity to neurologic structures and in preoperative planning.6 Single-photon emission CT can also

Management and Prognosis

Management of vertebral osteoid osteoma lesions without associated spinal deformity or neural element compression begins conservatively with anti-inflammatory medications. If this is unsuccessful, then minimally invasive techniques such as percutaneous radiofrequency thermal ablation (RFA) and laser photocoagulation effectively destroy the lesion.8 However, proximity of the lesion to neural structures may preclude the use of RFA, and use of these techniques may risk thermal damage to

adjacent neural structures. Vanderschueren et al8 reported success in 19 of 24 patients (79%) after 1 RFA treatment and in 23 of 24 patients (96%) after repeat RFA treatment. Spinal deformity persisted in three of seven (43%) patients after RFA treatment. En bloc excision is recommended in patients with osteoid osteoma with associated fixed spinal deformity, with neurologic compression, or if RFA is deemed unsafe due to the anatomic location or has been previously unsuccessful. Excision is curative and brings immediate relief of symptoms. If resection leads to spinal instability, then surgical excision with instrumentation and arthrodesis is recommended. Nonsurgical management of osteoid osteoma can lead to resolution of symptoms in 30 to 40 months,9 although pain can be severe and disabling, which can necessitate invasive treatment. With complete surgical excision, a cure is expected. Associated spinal deformity can resolve spontaneously if the lesion is resected within 15 months of onset of deformity.5

Osteoblastoma
Osteoblastoma is a benign boneforming neoplasm histologically similar to osteoid osteoma, but it is larger and clinically and histologically more aggressive. Osteoblastoma is rare, accounting for 1% to 5% of primary bone tumors; the spine accounts for 28% to 36% of osteoblastomas.10 Spinal osteoblasto-

Dr. Daniels or an immediate family member has received research or institutional support from Synthes and Flexuspine. Dr. Jonathan Schiller or an immediate family member serves as a paid consultant to or is an employee of DePuy. Dr. Czerwein or an immediate family member serves as a paid consultant to or is an employee of Stryker, Globus Medical, Medtronic Sofamor Danek, and Baxter. Dr. Terek or an immediate family member has stock or stock options held in Novartis, Pzer, Astrazeneca, and Johnson & Johnson and serves as a board member, owner, officer, or committee member of the Musculoskeletal Tumor Society. None of the following authors or any immediate family member has received anything of value from or has stock or stock options held in a commercial company or institution related directly or indirectly to the subject of this article: Dr. Thakur, Dr. Valdes, Dr. Alan Schiller, and Dr. Esmende.

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mas typically arise in the posterior elements, although extension into the vertebral body is common in larger tumors. They occur most often in the lumbar spine.10

Presentation
Spinal osteoblastoma presents with dull back pain or, due to its expansile nature, with symptoms from neural compression. Symptoms typically are not worse at night and respond poorly to nonsteroidal anti-inflammatory drugs. The average age at presentation is 20 to 24 years, although patients ranging from ages 1 to 72 years have been reported.2 Males are twice as commonly affected as females. Deficits in neural elements are reported in 32% of cases.11 Scoliosis is also known to occur in spinal osteoblastoma but less frequently than with osteoid osteoma.

is reddish brown due to its rich blood supply. Ten percent to 15% of osteoblastomas have an associated aneurysmal bone cyst (ABC) component. Histologically, osteoblastoma is similar to osteoid osteomas, with prominent osteoblasts that produce woven bone.2 Osteoblastoma can have a spectrum of histologic changes and may contain epithelioid osteoblasts.

primary bone tumors and 15% of all primary spine tumors.

Presentation
ABCs occur most commonly in the second decade of life, with a slight predominance toward females.14 Most patients report a slow, gradual onset of pain in the involved area, with an average of 24 weeks of symptoms before presentation. A palpable mass or spinal deformity may be present.

Management and Prognosis

Osteoblastomas are managed surgically because of both pain and the increasing size that can cause bony destruction, neural impingement, spinal deformity, or destabilization. Treatment consists of resection and, if resultant spinal instability is encountered, fusion. En bloc resection is preferred. If complete resection is not possible due to the anatomic location of the lesion, then intralesional curettage and cementation or bone grafting may be performed.10 Follow-up with radiographic imaging every 3 or 4 months for 2 years, then every 4 to 6 months until year 5, and yearly thereafter is recommended to monitor for recurrence. Prognosis is based on completeness of the resection, with a reported recurrence rate of 10% to 24%.10 Larger lesions and those locally more aggressive tend to have higher rates of recurrence. Poor patient outcome due to malignant transformation to sarcoma, spinal cord necrosis, and spinal cord compression are known to occur. Malignant conversion of osteoblastoma is rare, and the exact incidence is unknown, but cases have been reported.12

Evaluation and Pathology

Inflammatory markers (ie, erythrocyte sedimentation rate, C-reactive protein level) typically are not elevated. Plain radiographs, CT, and MRI are useful modalities in evaluating a possible ABC (Figure 2). In the study by Boriani et al,15 radiographic axial deformity was observed in >44% of patients. Most lesions are seen in the posterior elements of the spine, and nearly 70% of ABCs occur in the thoracolumbar region, often with multiple contiguous levels affected (30% to 40%).16 CT reveals a characteristic septate pattern representing a multilocular lytic lesion with cortical expansion and erosion17 (Figure 2). MRI is useful to evaluate neural impingement and soft-tissue involvement and may show multilocular lesions with fluidfluid levels on T2-weighted images; such multilocular lesions are highly indicative of ABCs.18 Angiography can be used as an adjunct to determine blood supply to the lesion and to identify possible arteriovenous shunts.19 The differential diagnosis of ABC includes malignant fibrous histiocytoma, giant cell tumor (GCT), and eosinophilic granuloma (EG). ABCs are often secondary lesions; hence, a thorough search for concurrent lesions such as GCT or osteoblastoma should be performed. Detection of

Evaluation and Pathology

Laboratory evaluation is typically normal. Radiographic evaluation is similar to that of osteoid osteomas, with the important distinction that the nidus is >20 mm in diameter. Osteoblastoma lesions commonly have multiple calcifications, aggressive bony destruction, and infiltration into surrounding tissues.2 Preoperative CT is useful for precisely defining the location of the tumor and extent of osseous involvement.11 The appearance on MRI is generally nonspecific and may overestimate the size of the lesion due to local inflammation and edema;11 however, MRI helps determine the effect of the tumor on neural elements as well as soft-tissue changes. Rarely, osteoblastoma can cause oncogenic osteomalacia, in which the serum phosphate level is low, calcium normal, and alkaline phosphatase elevated. On gross pathology, osteoblastoma
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Aneurysmal Bone Cyst

ABCs consist of a blood-filled cavity and have a lytic, expansile radiographic appearance.13 These lesions constitute approximately 1.4% of all

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Figure 2

Axial CT scan of an aneurysmal bone cyst in the lumbar spine. (Courtesy of Gagandeep Singh, radiopaedia.org, 2008. Accessed October 9, 2012.)

such lesions can change the anticipated nature of postoperative surveillance (eg, GCT is monitored for pulmonary metastases). Histologically, ABCs have large and/or small blood-filled spaces separated by septae. These septae consist of spindle-celled fibrous tissue and multinucleated osteoclast-like giant cells with thin trabeculae of woven lamellar bone without an endothelial lining. Sometimes osteoclasts are seen in the pools of blood. Blood vessels are usually thin-walled.16 The etiology of primary ABC in many cases is related to upregulation of ubiquitinspecific protease (USP6) gene.20

However, this is often not possible because of the location of the lesion. Moreover, en bloc tumor excision may lead to spinal instability; thus, spinal stabilization should be considered based on the degree of resection. Adjuvants such as cryosurgery, argon beam electrocautery, and polymethyl methacrylate cement have been described. One study demonstrated no recurrence in patients when argon beam electrocautery was used compared with a 33% recurrence in patients when it was not used.21 These adjuvant therapies should be employed intralesionally when there is no increased risk to the surrounding neural structures. There is little in the literature regarding nonsurgical management of spinal ABCs because early surgical intervention is the mainstay of treatment. Patients with preoperative neurologic deficits may see neurologic improvement postoperatively following decompression. Recurrence of the lesion is related to the extent of the resection. Intralesional resections have reported recurrence rates of up to 30%, whereas complete marginal resections can result in 10% recurrence rates.16 Most recurrences occur within the first 2 years after treatment. Patients should be monitored for 5 years, and those receiving radiation should have lifetime monitoring.15

growth. In hereditary multiple exostosis, multiple osteochondral lesions are found in the body, yet only 3% occur in the spinal column.22

Presentation
Patients present with pain and/or swelling in affected areas. The average age at presentation is 31 years, although patients with hereditary multiple exostosis may present earlier in life. 22 Neurologic manifestations from cord compression are rare.

Evaluation and Pathology

Laboratory studies are normal. Initial radiographic evaluation may reveal a spinal deformity, although radiographic diagnosis is sometimes challenging. The typical appearance of a lesion composed of mature cortical and medullary bone in continuity with the underlying bone is often not visualized. Sometimes a sessile or pedunculated bony mass may be observed. CT and MRI are recommended; they can be used to evaluate the connection of the medullary cavity of the lesion to the underlying bony segment. CT defines the osseous and cartilaginous margins effectively; MRI aids in determining the thickness of the cartilage cap (Figure 3). A cartilage cap of >3 cm thickness can indicate malignant transformation. The hallmark of an osteochondroma is the cartilage cap at the end of a stalk, which is attached to the parent bone without underlying cortex. Histologically, the cap is hyaline cartilage covered by dense collagen that constitutes the perichondrium. Endochondral ossification in the cap continues to occur until the patient achieves skeletal maturity.

Management and Prognosis

ABCs do not spontaneously resolve, and surgical management is the treatment of choice. Adjunct radiation therapy is not recommended because of the risk of developing radiation-induced myelopathy, spinal deformity, and sarcoma.13 Angiography can be used to embolize lesions preoperatively and reduce intraoperative blood loss. Intralesional curettage has a high recurrence rate (30%), and complete marginal resection is recommended.16

Osteochondroma
Osteochondroma is the most common benign adult bone tumor; however, only 1% to 4% of osteochondromas occur in the spine. Osteochondroma primarily occurs in the posterior elements. Almost 50% of these lesions occur in the cervical spine, primarily at C2, because of an osteocartilaginous proliferation at a growth plate. Osteochondromas stop increasing in size at the end of

Management and Prognosis

Symptomatic lesions or those causing neurologic deficit should be surgi-

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cally managed. Spinal instrumentation and arthrodesis to provide spinal stability should be considered if resection leads to instability.23 When possible, complete resection of the lesion is recommended. Complete resection of a lesion with benign pathology is typically curative. Malignant transformation of solitary osteochondromas is rare. In a series of 150 cases of solitary spinal osteochondromas, a malignant transformation rate of only 2.5% was reported.24 Lesions associated with multiple hereditary exostosis (MHE) have higher rates of malignant conversion.22

ing to weakness or paralysis. Fewer than 5% of patients with NF1 have symptomatic spinal involvement, whereas up to 38% may have asymptomatic lesions diagnosed by MRI.28 Spinal involvement is more common in patients with NF2 than in those with NF1.

Figure 3

Evaluation and Pathology

Laboratory studies are normal. Genetic testing helps determine the presence of NF1 or NF2, especially in patients with characteristic skeletal, cutaneous, and ophthalmologic manifestations. Radiographs may demonstrate sharp angular scoliosis, vertebral erosion or scalloping, and rib thinning. Enlargement of the intervertebral neuroforamina and hypoplasia of the pedicles may be seen. CT can differentiate vertebral destruction from soft-tissue expansion. Preoperative diagnosis of intradural extension by means of MRI is critical for surgical planning.26 Solitary neurofibromas are firm, rubbery masses usually without focal areas of softening and degeneration. They are well-defined but not encapsulated. Histologically, the tumors are composed of waxy, dark, thin nuclei embedded in thick irregular collagen fibers. Foci of myxoid stromal cells are usually present and may stain positive for S-100 protein. In neurofibromas associated with NF1 or NF2, tumor cells infiltrate surrounding tissue.

Neurobroma
Neurofibromas are tumors derived from Schwann cells. They are most commonly seen at the cervical and thoracic levels.25 They can occur in isolation or be associated with neurofibromatosis (NF) type 1 or NF2. They are often dumbbell-shaped as a result of constriction while exiting the neural foramen. Neurofibromas cause complications due to local growth and encroachment on surrounding neurovascular structures.

Lateral radiograph demonstrating osteochondroma (arrows) in the cervical spine.

Presentation
Neurologic impairment is commonly seen in older patients but is infrequently observed in young patients.26 Pain is the most common complaint.27 The spinal deformity that typically results is scoliosis and may be dystrophic or nondystrophic. A dystrophic curve is characterized by a short-segment (four to six vertebrae), sharply angulated deformity, often in the thoracic spine. Vertebral scalloping, enlarged foramina, and severe apical rotation can be seen. A nondystrophic curve is similar to an idiopathic scoliotic curve. Intraspinal neurofibromas may cause spinal cord or nerve root compression leadNovember 2012, Vol 20, No 11

Management and Prognosis

Symptomatic lesions should be excised en bloc if resection can be successfully performed without compromising critical neural structures. Noncritical involved nerve roots may be sacrificed.28 Hemilaminectomy is beneficial to preserve bone available for spinal stabilization and fusion.29 The intradural component may be successfully approached microsurgi-

cally to preserve the uninvolved nerve fascicles.30 Adjuvant therapy (ie, radiation, chemotherapy) has been used where tumor resection may be incomplete, especially in cases of NF2 in which malignant degeneration can occur. However, the use of adjuvant therapy is a matter of debate.24 Rapid progression of untreated dystrophic scoliosis carries a poor prognosis, making surgical management essential. Current recommendations suggest treating scoliosis aggressively with combined anterior and posterior fusion because bracing is ineffective. Constant surveillance is needed for nonunion or pseudarthrosis. Recurrence of neurofibromas, often secondary to incomplete resection, is a significant problem.31 Resection of the intraspinal portion of the tumor risks paraplegia and spinal

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cord injury, with up to 3% of patients suffering permanent dysfunction.31,32 Malignant degeneration can occur in untreated lesions, with a higher incidence reported in patients with neurofibromatosis than in those with sporadic lesions. Asymptomatic lesions do not require immediate resection, but close observation is recommended, and many lesions become symptomatic over time, thus requiring eventual resection.24,28

Giant Cell Tumor of Bone

GCT of bone accounts for 5% to 8% of all primary bone tumors, although only 2% to 5% of these lesions occur in the spine.33 GCT most often occurs in the vertebral body, with equal frequency in the cervical, thoracic, and lumbar regions.34 Females and males are affected with similar frequency; most patients present between the ages of 20 and 50 years. GCT lesions aggressively expand, making management difficult.

bone destruction. CT and MRI aid in defining bone, marrow, and softtissue involvement. Chest CT can aid in diagnosing pulmonary metastases. The radiographic differential diagnosis is brown tumor of hyperparathyroidism, metastases, hematologic malignancies, ABC, giant cell reparative granuloma, malignant fibrous histiocytoma, and chordoma. Open biopsy rather than image-guided biopsy is necessary to differentiate between these diagnoses, with the decision based on tumor location, size, and the treating physicians clinical judgment.34 Grossly, a GCT lesion is yellowtan, soft, and friable, often with areas of hemorrhage. Histologically, three findings define GCT: a random distribution of osteoclast-type giant cells, background oval or round mononuclear cells with a vesicular nucleus and poorly defined cytoplasm, and nuclei that are identical to those of mononuclear cells.

systemic diphosphonates, corticosteroids, interferon-, and, recently, a monoclonal antibody, Denosumab (Amgen, Thousand Oaks, CA), that blocks receptor activator of nuclear factor B ligand (RANKL).37 En bloc excision with wide margins has a local recurrence rate of 25%.35 However, with incomplete resections, recurrence rates are 30% to 50%, often necessitating re-excision or systemic treatment.34,38 When GCTs present in the spine/sacrum, metastases to the lungs occur in up to 13% of patients.39

Eosinophilic Granuloma
EG is a bone lesion resulting from Langerhans cell histiocytosis, which is a disease of the reticuloendothelial system that presents with spinal involvement in 10% to 15% of cases. These lesions occur usually in children aged <10 years, with a higher incidence in boys; adult cases have also been described.40,41 Lesions most often affect vertebral bodies in the thoracic spine, followed by the cervical and lumbar spine.42,43 The disease can be associated with systemic involvement seen in Hand-SchllerChristian disease or the severe, syndromic Letterer-Siwe disease.41 Multifocal spinal involvement is common and occurs in more than one third of cases of spinal EG.42,43

Management and Prognosis

En bloc excision with wide margins is the recommended treatment of thoracic and lumbar lesions, although neurologic deficit is a significant risk.34,35 In the cervical spine and sacrum, complete resection is often not possible and requires adjuvant therapy (ie, preoperative embolization, radiation, intralesional liquid nitrogen, phenol, polymethyl methacrylate).34 Lackman et al36 also demonstrated embolization as definitive management in sacral lesions. Several series have recommended adjuvant radiation for incomplete resection and local recurrences; however, the risk of malignant conversion is 25%.34 The complication rate with surgery can be significant, including massive bleeding, wound complications, and neurologic compromise. Other treatment modalities include

Presentation
Back pain is the most common presenting complaint, although radiating pain may occur. Typically, pain is present for months, which may lead to extensive involvement at the time of diagnosis. These expansile lesions can cause spinal cord compression and associated sequelae. In a series of 24 patients, all presented with pain, and half had neurologic deficits.34

Presentation
Patients with EG present with persistent back pain; restricted range of motion; deformity such as kyphosis, scoliosis, and/or torticollis; and, rarely, neurologic deficit.42,44 Diabetes insipidus is an associated extraskeletal abnormality resulting from pituitary gland involvement. Other sites that can be affected are the skin, lung, liver, spleen, and retro-orbital space.

Evaluation and Pathology

Laboratory evaluation is normal with GCT. Elevated serum calcium levels help distinguish brown tumor of hyperparathyroidism from GCT, which otherwise may be indistinguishable on microscopy. Radiographs reveal an expansile, lytic lesion without a sclerotic rim.35 Associated compression fractures may occur as a result of

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Figure 4

Lateral plain radiograph of vertebra plana. (Reproduced from Garg S, Dorman JP: Tumors and tumor-like conditions of the spine in children. J Am Acad Orthop Surg 2005;13[6]:372-381.)

nal spread or soft-tissue mass), then close follow-up is recommended, and unnecessary biopsy can be avoided.42 If atypical features are present (ie, soft-tissue mass, disk involvement, neurologic symptoms), then biopsy is indicated.42 Histologic examination of EG reveals numerous histiocytic cells (called Langerhans histiocyte), with a mixture of eosinophils, lymphocytes, and neutrophils. The Langerhans cell is S-100 proteinpositive; CD1a protein and CD68 antigen may also be positive. Intracytoplasmic tennis racquetshaped organelles, known as Birbeck granules, are seen with electron microscopy.

the lesion, followed by annual radiographs through skeletal maturity to monitor spinal balance and growth.

Hemangioma
Hemangiomas are vascular intraosseous lesions that can have extraosseous extension, most commonly occurring in the thoracic and lumbar spine. They are typically found incidentally through radiologic tests and are seldom symptomatic or aggressive. Hemangiomas are thought to occur in approximately 10% of all patients.46

Presentation
Symptoms are usually absent; only 1% of patients have symptomatic lesions. Symptoms typically consist of pain, with possible neurologic deficits. Aggressive hemangiomas have been reported to cause nerve root or spinal cord compression.47 Other presenting findings may include pathologic fractures, spontaneous epidural bleeding, and canal compromise. Deformity of the vertebrae with protrusion into the canal has also been described. Several rare medical syndromes have been described associated with spinal hemangiomas that may present in children. These include Cobb syndrome, Maffucci syndrome, Klippel-Trnaunay-Weber syndrome, Kasabach-Merritt syndrome, and McCune-Albright syndrome.

Management and Prognosis

EG of the spine often improves spontaneously, achieving variable degrees of vertebral body height reconstitution. As a result, surgery is rarely required.44 Management is usually symptomatic, with rest and analgesics/anti-inflammatory drugs. Orthoses are useful for pain and may help prevent kyphosis. Intralesional CT-guided steroid injection may be helpful for symptomatic solitary EGs of the spine. Low-dose radiation therapy has been used for cases with neurologic compromise; rarely, surgical curettage with stabilization may be needed if neurologic defects or spinal instability is observed. Chemotherapy may be used if EG is associated with systemic and/or multifocal disease; referral to an oncologist is appropriate for these individuals.42 Long-term prognosis is favorable, with extremely low rates of recurrence in skeletally immature patients. Recurrence is more likely in patients who are skeletally mature.40,43 Garg et al42 recommended close clinical follow-up with spinal radiographs every 3 months for the first year after diagnosis to confirm the benign nature of

Evaluation and Pathology

Laboratory evaluation should include a complete blood count, erythrocyte sedimentation rate, and measure of serum and urine osmolality if the patients history is positive for polyuria or polydypsia, suggesting diabetes insipidus. On radiographic evaluation, EG typically appears as lytic lesions in the vertebral body or, rarely, on the posterior elements. Approximately 40% of cases present as vertebra plana deformity (Figure 4), although asymmetric collapse of the vertebrae also occurs.40,42 Because of the high frequency of multiple skeletal lesions, skeletal survey or technetium Tc-99m bone scan is recommended to identify other affected sites.42 MRI helps confirm the diagnosis and can demonstrate associated soft-tissue mass. Differential diagnosis includes leukemia, lymphoma, infection, Ewing sarcoma, osteosarcoma, and ABC.42 Definitive confirmation of this lesion is obtained by biopsy.45 If radiographic and MRI findings are classically those for EG (ie, vertebral collapse, disk space maintained, no extraspiNovember 2012, Vol 20, No 11

Evaluation and Pathology

Laboratory examination is typically normal. Diagnosis is usually made with plain radiographs, which demonstrate the classic corduroy pattern with vertical striations (Figure 5). CT demonstrates punctuate sclerotic foci. Magnetic resonance images are hyperintense on both T1- and T2weighted sequences (Figure 6). In the

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Figure 5

Figure 6

Summary
Benign tumors of the spine are often incidental findings, although occasionally they cause deformity, nerve compression, or pain. A thorough understanding of the presenting symptoms, characteristics on imaging studies, need for biopsy, and available treatment modalities are necessary to ensure appropriate treatment of the patient. Some tumors generally require only observation (EG) or ablative treatment (osteoid osteoma, neurofibroma, hemangioma). Most, however, including osteoid osteoma, osteoblastoma, ABC, osteochondroma, neurofibroma, GCT of bone, and hemangioma, require surgery, typically following a period of nonsurgical management.

Lateral radiograph of a hemangioma demonstrating the classic corduroy pattern with vertical striations (arrow).

presence of progression of symptoms and/or neurologic deficit with structural changes, a biopsy may be necessary to differentiate a hemangioma from malignant forms of vascular tumors. Grossly, hemangioma is a dark, reddish, hypervascular lesion. Histologically, hemangiomas are characterized by a proliferation of numerous dilated blood vessels with associated flattened epithelium; this is in contrast to ABC lesions, which do not have an endothelial lining.

Sagittal T1-weighted magnetic resonance image demonstrating vertebral hemangioma at L3. (Courtesy of James O. Johnston, MD, University of San Francisco, CA. http://www.tumorlibrary.com.)

References
Evidence-based Medicine: Levels of evidence are described in the table of contents. Reference 4 is a level II study. References 5, 6, 10, 15, 27, 36, 38, 40-42, and 46 are level III studies. References 2, 3, 7-9, 11, 12, 16-19, 21-24, 28, 30-33, 35, 37, 39, 43-45, and 47-50 are level IV studies. References 13, 14, 25, 26, and 29 are level V expert opinion. References printed in bold type are those published within the past 5 years.
1. Unni KK, Inwards CY: Introduction and scope of study, in Dahlins Bone Tumors: General Aspects and Data on 10,165 Cases. Philadelphia, PA, Lippincott Williams & Wilkins, 2009. Jackson RP, Reckling FW, Mants FA: Osteoid osteoma and osteoblastoma: Similar histologic lesions with different natural histories. Clin Orthop Relat Res 1977;128:303-313. Swee RG, McLeod RA, Beabout JW: Osteoid osteoma: Detection, diagnosis, and localization. Radiology 1979;130(1): 117-123. Bottner F, Roedl R, Wortler K, Grethen C, Winkelmann W, Lindner N: Cyclooxygenase-2 inhibitor for pain

Management and Prognosis

Management is reserved for symptomatic lesions; however, determining whether the hemangioma is the cause of back pain requires careful consideration. Several options have been shown to be effective, including radiation therapy, intralesional ethanol injection, transarterial embolization, and vertebroplasty/kyphoplasty.48,49 For structurally aggressive lesions (ie, with pathologic fractures,

canal/nerve compromising lesions), surgery may be indicated. Prior to resection, careful preoperative planning requires angiography to identify the vascular source of the tumor as well as the spinal cord blood supply. Surgical intervention can include nerve decompression and reconstructive procedures, as needed. Hypervascularity of these tumors may predispose to surgical complications such as excessive blood loss or consumption coagulopathy. Patients with incidental lesions remain asymptomatic for many years; however, there are reports of lesions becoming painful after a period of quiescence. Lesions painful at presentation that require invasive treatment have had reported recurrence rates of 29% within 2 years of initial treatment.50 Postoperative clinical and radiographic follow-up is recommended to detect early recurrence.

2.

3.

4.

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