Osteoblastoma

DEFINITION AND PATHOGENESIS

• A rare benign tumor similar to osteoid osteoma histologically, but >2cm in size.

IMPORTANCE

• Osteosarcoma must not be misdiagnosed as osteoblastoma
• 0.8% of all bone tumors (osteosarcoma is 20X more frequent)

CLINICAL FEATURES

• Pain (87%)(often nocturnal and not relieved with ASA), swelling, pathologic fracture
• 80% between 10-30 years of age, and 90% <40 years (similar to the age distribution of osteosarcoma)
• M:F = 2-3:1
• Scoliosis, possible cord and root compressive symptoms
• ??gait disturbance
• Rarely alk? is ?
• Reported as a cause of osteomalacia

RADIOLOGIC FEATURES

• 30-40% occur in the spine and sacrum(55% in posterior and 42% in anterior and posterior elements)
  • Round with expansile component
  • Thin periosteal reactive new bone
  • Bone forming matrix or densities
  • X-rays may not show lesion
• Location
  • ?34% occur in long bones
  • ?15% occur in skull, mandible, maxilla
  • ?10% occur in hands/feet
  • Unusual location: carpal bones
• Lytic to blastic with fusiform bone expansion surrounded by a thin reactive host bone sclerosis
• Usually central in metadiaphysis, rarely juxtacortical
• Cortical erosion to complete focal destruction
• Soft tissue mass
• Soft tissue margins after en bloc resection
• Extension through open epiphyseal plate
• Extension to joint
• Vascular and/or LN involvement
• Skip metastases have been reported

GROSS PATHOLOGY

• May be extremely vascular and deep red grossly to white with intense bone production
• Gritty and friable to hard
• Cortical bone expanded, thinned, or totally destroyed
• May be subperiosteal, cortical, or medullary in location

HISTOLOGIC FEATURES

• As in osteoid osteoma: woven bone within loose stroma
• Must distinguish from osteosarcoma
  • (1) No anaplasia (rarely pseudoanaplasia in"pseudomalignant" osteoblastoma)
  • (2) Osteoblastic rimming is present
  • (3) No cartilage
  • (4) No permeation or trabecular trapping of host bone by the tumor
  • (5) Mitotic activity is prominent
• Osteoclast numbers are variable
• Think osteosarcoma if:
  • (1) Long spindle cells in sheets
  • (2) Stroma between osseous trabeculae is not free of osteoid
  • (3) Solid wall of stroma
  • (4) Cartilage being produced (rarely can be present in osteoblastoma)

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

• Osteosarcoma
• Osteoid osteoma
• Giant cell tumor
• Aneurysmal bone cyst

DISEASE COURSE AND TREATMENT

• Biopsy at the margin of the lesion for accurate representative sample
• Recurrence rare with complete excision and should point to possibly an initial misdiagnosis
• Intralesional curettage, aided by high speed burr and adjuvant cryotherapy can result in complete excision
• En bloc resection in expendable bones such as ribs or fibula
  • Has been reported for calcaneal lesions
• XRT or even chemotherapy may be necessary to control spinal lesions
• Preoperative selective arterial embolization may be helpful in vertebral or pelvic locations
• Watch for XRT induced malignancies (eg, fibrosarcoma) in spine lesions
• Lung metastases reported

SPECIAL CONSIDERATIONS

• TOXIC OSTEOBLASTOMA
  • Massive systemic, immune response to the lesion
• PERIOSTEAL OSTEOBLASTOMA
• PSEUDOMALIGNANT OSTEOBLASTOMA
  • Bizarre appearance histologically represents degeneration of nuclei
• AGGRESSIVE OSTEOBLASTOMA
  • Most prominent feature is "epithelioid" osteoblasts, high recurrence, no metastases
• MULTIFOCAL OSTEOBLASTOMA
  • Reported in multiple bones of a single extremity
  • Multiple lytic skeletal lesions
  • Ddx includes vascular neoplasia, metastases, multiple myeloma, multicentric osteosarcoma

REFERENCES

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Scoccianti G, Campanacci DA, Innocenti M, Beltrami G, Capanna R.: Total calcanectomy and reconstruction with vascularized ilia bone graft for osteoblastoma: a report of two cases. Foot Ankle Int. 2009 Jul;30(7):716-20.

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Bone Island (Enostosis)

Osteoid Osteoma