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Updated: Jun 21 2021

Periosteal Osteosarcoma

Images
https://upload.orthobullets.com/topic/8016/images/Case A - tibia - xray - Parsons_moved.png
https://upload.orthobullets.com/topic/8016/images/Case A - tibia - MRI - Parsons_moved.png
https://upload.orthobullets.com/topic/8016/images/Histology B_moved.png
https://upload.orthobullets.com/topic/8016/images/osteosarcoma clinical picture.jpg
https://upload.orthobullets.com/topic/8016/images/osteosarcoma radiograph sunburst periosteal reaction.jpg
https://upload.orthobullets.com/topic/8016/images/Histology D_moved.jpg
  • summary
    • Periosteal Osteosarcomas are rare, malignant, intermediate-grade, surface osteosarcomas that occur most commonly on the diaphysis of the femur and tibia. Patients typically present between the ages of 15 to 25 years with regional pain and swelling. 
    • Diagnosis is made with radiographs showing a lesion that has a classic "sunburst or "hair on end" periosteal reaction with biospy showing cellular atypia with areas of osteoid and chondroblastic matrix.
    • Treatment is usually neo-adjuvant chemotherapy, limb salvage surgical resection, followed by adjuvant chemotherapy. 
  • Epidemiology
    • Incidence
      • extremely rare intermediate grade surface osteosarcoma
    • Demographics
      • usually occurs in patients 15 to 25 years of age
      • more common in females than males
    • Anatomic location
      • occurs most commonly in the diaphysis of long bones
      • femur and tibia are most common
  • Etiology
    • Genetics
      • germ-line mutation of p53 found in 15-20% of cases
  • Presentation
    • Symptoms
      • pain is the most common presenting symptom
      • 25% present with pathologic fracture
    • Physical exam
      • regional swelling and tenderness
  • Imaging
    • Radiographs
      • lesion has a classic "sunburst" or "hair on end" periosteal reaction
        • often sunburst periosteal reaction occurs in a saucerized cortical depression
      • typically there is no involvement of the medullary canal
    • Chest CT scan
      • required for staging
      • evaluates for the presence of pulmonary metastasis
    • Bone scan
      • required for staging
      • usually very hot
  • Histology
    • Classically
      • grossly tumor appears lobular and cartilaginous
      • while tumor produces osteoid, histology reveals areas of chondroblastic matrix
        • if histology shows no osteoid production, tumor would be classified as a chondrosarcoma
  • Treatment
    • Operative (same as intramedullary osteosarcoma)
      • multi-agent chemotherapy and limb salvage resection
        • indications
          • standard of care in most patients
        • chemotherapy
          • preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 6-12 months after surgical resection
        • surgical resection
          • trend towards limb salvage whenever possible
  • Prognosis
    • 20-35% chance of pulmonary metastasis
    • Intermediate prognosis between parosteal and intramedullary osteosarcoma
    • 90-98% necrosis with neoadjuvant chemotherapy is good prognostic sign
    • Expression of multi-drug resistance (MDR) gene portends very poor prognosis
      • cells can pump chemotherapy out of cell
      • present in 25% of primary lesions and 50% of metastatic lesions
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