Benign bone tumors


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Benign bone tumors.

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Benign bone tumors

  1. 1. Benign Bone Tumors DR.BASIT@LIVE.COM
  2. 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. 3. Evaluation  History  Examination  Radiographic studies  Plain  Computed tomography  Magnetic resonance imaging  Scintigraphy
  4. 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. 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. 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. 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. 8. Management  Observation  Curettage and bone grafting  Excision
  9. 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. 10. Osteoid osteoma
  11. 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. 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. 13.  25 percent of osteoid osteomas are not obvious on plain radiographs  Small, round lucency (nidus) with a sclerotic margin  Central ossification Radiography
  14. 14.  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
  15. 15.  Asymptomatic  Observed with serial examinations and radiographs every four to six months  Symptomatic  Radiofrequency ablation  Surgical resection Treatment
  16. 16. Osteoblastoma
  17. 17.  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
  18. 18.  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
  19. 19. 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
  20. 20. Differential diagnosis  Stress fracture  Infection (eg, osteomyelitis, bone abscess)  Osteoid osteoma  Osteosarcoma, a malignant bone tumor  Aneurysmal bone cyst
  21. 21. Treatment  Curettage and bone grafting  En block excision  Radiation
  22. 22. Prognosis  Good, if the lesion can be completely removed  Rate of recurrence is up to 20% if the lesion has expanded outside the bone
  23. 23. Osteochondroma Hereditary multiple osteochondromas
  24. 24.  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
  25. 25.  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
  26. 26.  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
  27. 27. 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
  28. 28. Differential diagnosis  Parosteal osteosarcoma  Medullary canal of osteochondromas is always continuous with that of the bone
  29. 29. 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
  30. 30. Enchondroma
  31. 31.  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
  32. 32.  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
  33. 33. 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
  34. 34. 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
  35. 35. Treatment  Observation  Asymptomatic  Without increased risk of pathological fracture  Curettage and bone grafting  Fractures should be permitted to heal before curettage
  36. 36. 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
  37. 37. Periosteal chondroma
  38. 38.  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
  39. 39. 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
  40. 40. Differential diagnosis  Nonossifying fibroma  Soft-tissue tumors, secondarily eroding into the cortical bone  Chondrosarcoma, a malignant tumor  Osteosarcoma, a malignant tumor
  41. 41. Treatment  Extended curettage  En block excision
  42. 42. Chondroblastoma
  43. 43.  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
  44. 44. Radiographic findings  Small, well-defined lesions with a sclerotic border that may cross the physis  Matrix calcification may be seen
  45. 45. 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
  46. 46. Treatment  Curettage and bone grafting
  47. 47. Prognosis  The prognosis is generally good.  Recurrence rates of up to 20 percent are reported
  48. 48. Chondromyxoid fibroma
  49. 49.  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
  50. 50. Radiographic findings  Eccentric, intramedullary, lobulated or bubbly lesion in the metaphysis  Sclerotic border
  51. 51. Differential diagnosis  Nonossifying fibroma  Aneurysmal bone cyst  Chondroblastoma  Osteomyelitis  Fibrous dysplasia
  52. 52. Treatment  Curettage and bone grafting
  53. 53. Prognosis  The prognosis is generally good  20 percent risk of recurrence
  54. 54. Fibrous dysplasia
  55. 55.  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
  56. 56.  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
  57. 57. 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
  58. 58. Differential diagnosis  Nonossifying fibroma  Unicameral bone cyst  Aneurysmal bone cyst  Chondromyxoid fibroma
  59. 59. Treatment  Asymptomatic  Observation  Symptomatic  Curettage, bone grafting  Autograft should not be used because it will be resorbed  Bisphosphonate therapy
  60. 60. 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
  61. 61. Ossifying fibroma (Jaffe-Campanacci syndrome)
  62. 62.  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
  63. 63. Radiographic findings  Lytic thinning of the diaphyseal cortical bone with interspersed sclerosis, causing anterior or anterolateral bowing  Sharply circumscribed margin
  64. 64. Differential diagnosis  Monostotic fibrous dysplasia (which originates in the medulla rather than the cortex)  Adamantinoma (a low-grade malignant bone tumor)  Nonossifying fibroma.
  65. 65. Treatment  Asymptomatic  Observation  Symptomatic  Excision, bone graft, and correction of bony deformity
  66. 66. Prognosis  Noninvasive  Recurs if excised before skeletal maturity
  67. 67. Nonossifying fibroma
  68. 68.  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
  69. 69. Radiographic findings  Small, well-defined, eccentric, expansile, lytic lesions located in the metaphysis with scalloped sclerotic borders
  70. 70. Differential diagnosis  Chondromyxoid fibroma  Fibrous dysplasia  Langerhans cell histiocytosis
  71. 71. Treatment  Asymptomatic  Nonossifying fibromas that are discovered incidentally do not require any further follow-up  Symptomatic  Curettage and bone grafting
  72. 72. Prognosis  Generally excellent  Usually fill in during adolescence  Risk of recurrence is lower than for other benign tumors
  73. 73. Unicameral bone cysts
  74. 74.  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
  75. 75. 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
  76. 76. Differential diagnosis  Aneurysmal bone cyst  Fibrous dysplasia
  77. 77. 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
  78. 78. Prognosis  Spontaneously resolve in all patients  Resolution may not occur until after skeletal maturity
  79. 79. Aneurysmal bone cyst
  80. 80.  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
  81. 81.  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
  82. 82. 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
  83. 83. 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)
  84. 84. Treatment  Excision  Curettage, and bone grafting  Chemical cauterization or cryotherapy may be required.
  85. 85. Prognosis  Continue to expand until treated  May recur after excision (in 10 to 50 percent of cases)
  86. 86. Langerhans cell histiocytosis
  87. 87.  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
  88. 88.  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
  89. 89. 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
  90. 90. Treatment  Asymptomatic  Conservative treatment  Symptomatic  Steroid injection  Curettage and bone grafting  Vertebra plana  Conservative  Temporary bracing  Radiation  Surgical decompression and fusion with instrumentation
  91. 91. Prognosis  Overall prognosis for skeletal disease is excellent  Low rate of local recurrence
  93. 93.  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
  94. 94.  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
  95. 95. 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
  96. 96. 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
  97. 97. Treatment  Curettage and bone grafting  En-bloc resection