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Updated: Jun 22 2021


Images A - tibia - xray a - parsons_moved.jpg A - femur - xray b - Parsons_moved.gif A - femur - xray a - Parsons_moved.gif B - tibia - xray B - Parsons_moved.png A - femur - MRI T1 - Parsons_moved.gif A - tibia - xray b - parsons_moved.jpg A - Tibia - T1 - parsons_moved.jpg B - tibia - xray - parsons_moved.png[1]_moved.jpg A_moved.jpg histo.jpg
  • summary
    • Adamantinomas are rarelow-grade malignant tumors of unknown etiology that are almost always located in the diaphysis of the mid-tibia.
    • The condition usually presents in patients who are between 20 and 40 years old with regional pain and a palpable mass.
    • Diagnosis is made with a biopsy showing nests of epithelial-like cells arranged in palisading or glandular pattern, in a background of fibrous stroma.
    • Treatment is usually wide-margin surgical resection.
  • Epidemiology
    • Incidence
      • less than 300 cases have been documented
    • Demographics
      • occurs in young adults (20 - 40 years of age)
    • Anatomic location
      • almost always located in mid-tibia 
  • Etiology
    • Pathophysiology
      • unknown
    • Associated conditions
      • osteofibrous dysplasia
        • historically, it was thought that osteofibrous dysplasia (OFD) was a precursor to this adamantinoma, however current studies have cast doubt on this theory
  • Presentation
    • Symptoms
      • pain of months to years duration
    • Physical exam
      • bowing deformity or a palpable mass of tibia is common
  • Imaging
    • Radiographs
      • multiple sharply circumscribed lucent lesions ("soap bubble" appearance) with interspersed sclerotic bone in mid-tibia
        • some lesions may destroy cortex
      • may see bowing of the tibia
      • radiographic evolution of lesions is helpful in the diagnosis as lesions may continue to grow and erode thru the cortex
      • unlike other primary bone tumors, adamantinoma typically shows no periosteal reaction
  • Studies
    • Histology
      • biphasic
      • contains both epithelial and fibrous mesenchymal cells
        • nests of epithelial-like cells arranged in palisading or glandular pattern
          • stain for keratin
        • background of fibrous stroma
  • Differential
    • Osteofibrous dysplasia
      • differentiating between osteofibrous dysplasia and adamantinoma is critical
        • osteofibrous dysplasia is benign and treated with observation
        • adamantinoma is malignant and treat with surgical resection
      • Differential diagnosis of Adamantinoma
      • Tibial diaphysis lesion
      • Treated with wide-resection alone
      • Adamantinoma
      • o
      • o
      • Osteofibrous dysplasia
      • o
      • Chondrosarcoma
      • o
      • Parosteal osteosarcoma
      • o
  • Treatment
    • Operative
      • wide-margin surgical resection
        • indications
          • standard of care in most patients
        • techniques
          • often requires intercallary resection with allograft or intercallary megaprosthesis reconstruction
          • as adamantinoma is a low-grade malignancy, radiotherapy and/or chemotherapy is not typically used for local control of disease
  • Prognosis
    • May metastasize to lungs (25%), therefore long-term followup is recommended
    • Recurrence is uncommon with negative margin excision
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