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Benign bone tumors
- 2. Range form static lesions to locally aggressive lesions May be diagnosed on plain radiographs Asymptomatic Incidental finding Symptomatic Pain Swelling Deformity Pathological fracture
- 3. Evaluation History Examination Radiographic studies Plain Computed tomography Magnetic resonance imaging Scintigraphy
- 4. Plain radiographs Best initial modality Should include views in 2 planes 80 – 90 % of cases can be diagnosed Advanced imaging should not be necessary for clearly benign lesions
- 5. Plain radiographs Where is the tumor? (Long bone or flat bone?; epiphysis, metaphysis, or diaphysis?; medulla, cortex, or surface?) What is the tumor doing to the bone? Is the tumor destroying or replacing existing bone? If so, what is the pattern? What is the bone doing to the tumor? Is there periosteal or endosteal reaction? If so, is it well-developed? Is it sharply defined? What is the type of periosteal reaction: reinforcing, spiculated, solid, interrupted? Are there intrinsic characteristics that suggest histology? Is there bone formation? Calcification? Is the lesion completely radiolucent?
- 6. Benign lesions Well-defined or sclerotic border Sharp zone of transition Small size or multiple lesions Confinement by natural barriers (eg, growth plate, cortex) Lack of destruction of the cortex Lack of extension into the soft tissue
- 7. Aggressive lesions Poor definition Cortical destruction ("moth-eaten" or permeative pattern) Spiculated or interrupted periosteal reaction Extension into the soft tissue Large size The absence of these findings does not exclude an aggressive lesion
- 8. Management Observation Curettage and bone grafting Excision
- 9. Classification Bone-forming tumors Osteoid osteoma Osteoblastoma Cartilage-forming tumors Osteochondroma Chondroma Chondroblastoma Chondromyxoid fibroma Fibrous lesions Fibrous dysplasia Ossifying fibroma Nonossifying fibroma Cystic and vascular lesions Unicameral bone cyst Aneurysmal bone cyst
- 10. Osteoid osteoma
- 11. Presents during the second decade Proximal femur is the most common site Progressively increasing pain that is worse at night and unrelated to activity Pain is relieved by aspirin or other nsaids, usually within 20 to 25 minutes
- 12. Children with lower-extremity lesions Limp, swelling, muscular atrophy, leg-length discrepancy, bone deformities, muscle contractures, and local point tenderness Children with spine lesions Limp, scoliosis, localized tenderness, restriction of motion, and/or spasm of paravertebral muscles
- 13. 25 percent of osteoid osteomas are not obvious on plain radiographs Small, round lucency (nidus) with a sclerotic margin Central ossification Radiography
- 15. Stress fracture The pain of stress fractures usually worsens with activity and is relieved with rest Plain radiographs, stress fractures typically are linear and run perpendicular or at an angle to the cortex, rather than parallel to it Bone infections May have a tract that extends from the lesion toward the nearest growth plate Osteoblastoma The pain is more generalized and chronic and less responsive to nsaids. It typically has a larger nidus, although this may not be visible Differential diagnosis
- 16. Asymptomatic Observed with serial examinations and radiographs every four to six months Symptomatic Radiofrequency ablation Surgical resection Treatment
- 17. Osteoblastoma
- 18. Rare benign bone-forming tumor of unknown etiology Presents during the second decade Common location is the posterior column of the spine Tumors in the spine may be difficult to identify on plain radiographs
- 20. Patients with osteoblastoma typically complain of chronic pain less responsive to nsaids Children with spine lesions Limp or neurologic symptoms Children with lower extremity lesions Limp
- 21. Radiography The radiographic findings are variable Advanced imaging (eg, CT or MRI) often is required for identification Appear similar to osteoid osteoma but is usually larger (>2 cm in diameter) May appear as an expansive lesion, similar to an aneurysmal bone cyst Rarely extend into the soft tissues
- 23. Differential diagnosis Stress fracture Infection (eg, osteomyelitis, bone abscess) Osteoid osteoma Osteosarcoma, a malignant bone tumor Aneurysmal bone cyst
- 24. Treatment Curettage and bone grafting En block excision Radiation
- 25. Prognosis Good, if the lesion can be completely removed Rate of recurrence is up to 20% if the lesion has expanded outside the bone
- 27. Present during the second decade Around the knee or the proximal humerus Distal femur is the most common location Osteochondroma Bony spur arising on the external surface of a bone covered by cartilaginous cap
- 29. Hereditary multiple osteochondromas (HMO) Two or more exostoses in the appendicular and axial skeleton Autosomal dominant inheritance of a germline mutation in the tumor suppressor genes EXT1 or EXT2 Prevalence in the general population is approximately 1:50,000 Painless mass near a joint or on the axial skeleton Painful mass associated with local trauma
- 30. Osteochondromas near the ends of long bones are palpable Osteochondroma can affect nearby growth plates Can involve the vertebra and may encroach on the spinal canal HMO may have short stature and angular deformities Osteochondromas grow throughout childhood They stop growing when the physes (growth plates) close and remain static throughout adulthood
- 31. Radiography Bony spur (sometimes large) that arises from the surface of the cortex and usually points away from the joint The cortex of the spur is continuous with the cortex of the underlying bone The cartilage cap is thick in the child (may be >2 cm), narrows during adolescence, and generally is <1 cm in the adult Biopsy and removal of the entire osteochondroma may be warranted for lesions with a cap ≥2 cm thick
- 32. Differential diagnosis Parosteal osteosarcoma Medullary canal of osteochondromas is always continuous with that of the bone
- 33. Treatment Can be observed without treatment Indications for excision Local irritation Deformity and concern for malignant transformation Prognosis Moderate risk of recurrence if osteochondromas are removed before the physes close Small lifetime risk of malignant transformation to chondrosarcoma Osteochondromas of the spine, scapula, pelvis, and proximal femur are particularly prone to malignant transformation
- 34. Enchondroma
- 35. Benign cartilage-forming tumors that develop in the medulla (marrow cavity) of long bones Enchondromas typically present during the second decade Enchondromatosis (ollier disease) is defined by multiple enchondromas, often with a unilateral predominance Enchondromatosis usually presents in children younger than 10 years Maffucci syndrome is a subtype of enchondromatosis that is characterized by multiple enchondromas and soft tissue hemangiomas
- 37. The signs and symptoms vary depending upon the anatomic site, extent, and distribution of involvement Asymptomatic unless a fracture is present Ncidental findings Symptomatic Widening of the bone, angular deformity, and limb-length discrepancy
- 38. Radiographic findings Oval, well-circumscribed, central lucent lesion, with or without matrix calcifications Expansion of the surrounding cortex, especially when the lesion is in the hand or foot Multiple lesions may be present
- 39. Differential diagnosis Bony infarcts Calcification is mainly in the periphery of the lesion and has a wavy or serpentine appearance Low-grade chondrosarcoma Pain without fracture
- 40. Treatment Observation Asymptomatic Without increased risk of pathological fracture Curettage and bone grafting Fractures should be permitted to heal before curettage
- 41. Prognosis Solitary enchondromas usually are self-limited Recurrence after curettage and bone graft is rare Malignant transformation of a solitary enchondroma is extremely rare (<1 percent) The risk of malignant transformation is increased (as high as 20 to 50 percent) in patients with enchondromatosis (Ollier disease) or Maffucci syndrome
- 43. Rare, benign, cartilage-forming tumors that arise from the surface of the cortex, deep in the periosteum, and erode into the cortex Occurs in children and adults Most common site is the proximal humerus Pain at the site of the lesion Palpable nontender hard mass that is fixed to bone
- 44. Radiographic features Small, scalloped, radiolucent lesions on the outer surface of the cortex in the metaphysis or diaphysis Rim of sclerotic bone Calcification is present in approximately one-third of cases Periosteal reaction is minimal
- 46. Differential diagnosis Nonossifying fibroma Soft-tissue tumors, secondarily eroding into the cortical bone Chondrosarcoma, a malignant tumor Osteosarcoma, a malignant tumor
- 47. Treatment Extended curettage En block excision
- 48. Chondroblastoma
- 49. Arises in the epiphyses or apophyses of long bones Presents during the teenage years The most common sites are the epiphysis of the proximal humerus, distal femur, and proximal tibia Low-grade joint pain (constant, unrelated to activity) and swelling
- 50. Radiographic findings Small, well-defined lesions with a sclerotic border that may cross the physis Matrix calcification may be seen
- 52. Differential diagnosis Giant cell tumor Benign but locally aggressive skeletal tumor that occurs near the growth plate in young adults Chondromyxoid fibroma Avascular necrosis Abnormality of subchondral bone in which pain is activity related. In contrast, in chondroblastoma, subchondral bone is normal, and pain is constant, unrelated to activity Aneurysmal bone cyst Osteomyelitis Clear cell chondrosarcoma
- 53. Treatment Curettage and bone grafting
- 54. Prognosis The prognosis is generally good. Recurrence rates of up to 20 percent are reported
- 56. Rare, benign, cartilage-forming tumor of the tubular long bones Usually presents in the teens or 20s One-quarter of cases occur in the proximal tibia Pain and swelling
- 57. Radiographic findings Eccentric, intramedullary, lobulated or bubbly lesion in the metaphysis Sclerotic border
- 59. Differential diagnosis Nonossifying fibroma Aneurysmal bone cyst Chondroblastoma Osteomyelitis Fibrous dysplasia
- 60. Treatment Curettage and bone grafting
- 61. Prognosis The prognosis is generally good 20 percent risk of recurrence
- 63. Lesion in which portions of the bone are replaced by fibrous connective tissue and poorly formed trabecular bone Originates in the medullary cavity Postzygotic mutation in the guanine nucleotide stimulatory protein (GNAS1) gene May occur in single or multiple bones The polyostotic form of fibrous dysplasia is known as mccune- albright syndrome and is associated with endocrine abnormalities and café-au-lait spots Mazabraud syndrome is characterized by fibrous dysplasia and soft tissue myxomas
- 65. Presents in the teens or 20s Most common in the proximal femur tibia, ribs, and skull Most patients with fibrous dysplasia are asymptomatic May be painful or cause swelling Repeated pathologic fractures or severe bone deformity "Shepherd's crook" varus deformity of the proximal femur
- 67. Radiographic findings Lytic lesion in the metaphysis or diaphysis with a "ground glass" appearance Expansion of the bone and possible bowing Cortical bone is thinned with a scalloped, undulating pattern due to endosteal erosion Periosteal reaction usually is absent unless there is a pathologic fracture
- 69. Differential diagnosis Nonossifying fibroma Unicameral bone cyst Aneurysmal bone cyst Chondromyxoid fibroma
- 70. Treatment Asymptomatic Observation Symptomatic Curettage, bone grafting Autograft should not be used because it will be resorbed Bisphosphonate therapy
- 71. Prognosis Deformity may progress with skeletal growth Usually is static after growth ceases but may be reactivated with pregnancy Often recurs after curettage and bone grafting
- 73. Deformity-inducing fibro-osseous lesion of the tibia and/or fibula Originates in the cortex Occurs in children younger than 10 years of age Swelling and/or anterolateral bowing of the lower leg Painful only if it associated with a pathologic fracture
- 74. Radiographic findings Lytic thinning of the diaphyseal cortical bone with interspersed sclerosis, causing anterior or anterolateral bowing Sharply circumscribed margin
- 75. Differential diagnosis Monostotic fibrous dysplasia (which originates in the medulla rather than the cortex) Adamantinoma (a low-grade malignant bone tumor) Nonossifying fibroma.
- 76. Treatment Asymptomatic Observation Symptomatic Excision, bone graft, and correction of bony deformity
- 77. Prognosis Noninvasive Recurs if excised before skeletal maturity
- 79. Developmental defect filled with fibrous connective tissue Known as metaphyseal cortical defect, fibrous cortical defect, and benign metaphyseal bone scar Incidental radiographic finding in teenagers Most commonly in the distal femur, followed by the distal tibia, and the proximal tibia Large lesions may be associated with pathologic fracture
- 80. Radiographic findings Small, well-defined, eccentric, expansile, lytic lesions located in the metaphysis with scalloped sclerotic borders
- 82. Differential diagnosis Chondromyxoid fibroma Fibrous dysplasia Langerhans cell histiocytosis
- 83. Treatment Asymptomatic Nonossifying fibromas that are discovered incidentally do not require any further follow-up Symptomatic Curettage and bone grafting
- 84. Prognosis Generally excellent Usually fill in during adolescence Risk of recurrence is lower than for other benign tumors
- 86. Fluid-filled lesions with a fibrous lining Generally occur in the first 20 years of life Proximal humerus and femur are the most common locations Commonly present with a pathologic fracture May be an incidental radiographic finding Localized pain, limp, or failure to use the extremity normally
- 87. Radiographic findings Well-marginated cystic lesions of the metaphysis or metadiaphysis without reactive sclerosis Usually involves the full diameter of bone, with expansion of the cortex "Fallen fragment" or "fallen leaf" sign
- 89. Differential diagnosis Aneurysmal bone cyst Fibrous dysplasia
- 90. Treatment Observation with serial radiographs Activity restrictions to avoid pathologic fracture Aspiration and injection with methylprednisolone Curettage and bone grafting rarely are required for large lesions that compromise the structural integrity of the bone
- 91. Prognosis Spontaneously resolve in all patients Resolution may not occur until after skeletal maturity
- 93. Expansile vascular lesions that consist of blood-filled channels May grow rapidly and destroy bone Generally are solitary Primary or related to other benign bone lesions (eg, giant cell tumor, osteoblastoma, chondroblastoma) Generally occur in adolescents
- 94. May be found in any bone Most common in the posterior spinal elements, femur, and tibia Typically cause localized pain Present with pathologic fracture, limp, or swelling, neurologic symptoms Lesions that cross the growth plate may cause growth arrest
- 95. Radiographic findings Aggressive, expansile, lytic metaphyseal lesions with an "eggshell" sclerotic rim Pathologic fracture or periosteal reaction may be present Sharply circumscribed "Soap bubble" appearance secondary to the reinforcement of the remaining trabeculae that support the bone structure The cortex is usually intact
- 97. Differential diagnosis Unicameral bone cyst Giant cell tumor, a benign but locally aggressive skeletal tumor that occurs in young adults Osteosarcoma, a malignant bone tumor Osteoblastoma (in the spine). Chondroblastoma (if they cross the growth plate)
- 98. Treatment Excision Curettage, and bone grafting Chemical cauterization or cryotherapy may be required.
- 99. Prognosis Continue to expand until treated May recur after excision (in 10 to 50 percent of cases)
- 101. Eosinophilic granuloma of bone Relatively rare disorder of unknown etiology Probably arising from circulating myeloid dendritic cells Most common in children 5 to 10 years of age Hand-schüller-christian disease classically refers to the clinical triad of skull lesions, exophthalmos, and diabetes insipidus Presentation of EGB with a single bone lesion is more common than multiple bone lesions
- 102. Bones most commonly affected in children include the skull, ribs, pelvis, long bones, mandible, and vertebrae. Patients with egb present with painful swelling at the affected bony site with or without decreased range of motion. Pathologic fracture or spinal cord compression may also occur. Biopsy of suspicious lesions and staining for cd1a and/or anti- langerin (cd207) is needed in order to confirm the diagnosis of egb Electron microscopy to identify birbeck granules is performed less frequently
- 103. Radiographic findings Well-defined, lytic lesion, with or without sclerotic margins, in the diaphysis or metaphysis Periosteal reaction may be absent, benign, or aggressive-appearing Associated soft tissue mass may be present Marked flattening of the vertebral body, or vertebra plana, is a common manifestation
- 105. Treatment Asymptomatic Conservative treatment Symptomatic Steroid injection Curettage and bone grafting Vertebra plana Conservative Temporary bracing Radiation Surgical decompression and fusion with instrumentation
- 106. Prognosis Overall prognosis for skeletal disease is excellent Low rate of local recurrence
- 107. GIANT CELL TUMOR
- 108. 5% of bone neoplasms Typically occur in patients 20 to 40 years old Most common location for this tumor is the distal femur, followed closely by the proximal tibia and distal radius Spinal involvement, other than the sacrum, is rare Usually solitary lesions Although these tumors typically are benign, pulmonary metastases occur in approximately 3% of patients
- 109. The overall mortality rate from disease for patients with pulmonary metastases is approximately 15% Progressive pain that often is related to activity initially and only later becomes evident at rest In 10% to 30% of patients, pathological fractures are evident at initial examination
- 110. Radiographic findings Eccentrically located in the epiphyses of long bones and usually abut the subchondral bone The lesions are purely lytic Zone of transition can be poorly defined on plain radiographs Partial rim of reactive bone may be present Frequently expands or breaks through the cortex Intraarticular extension is rare On MRI, the lesion usually is dark on t1-weighted images and bright on t2-weighted images
- 111. CLASSIFICATION Grade I Intraosseous lesions with well-marginated borders and an intact cortex Grade II More extensive intraosseous lesions having a thin cortex without loss of cortical continuity Grade III Extraosseous lesions that break through the cortex and extend into soft tissue
- 112. Treatment Curettage and bone grafting En-bloc resection