Periosteal Osteosarcoma

Topic updated on 04/28/13 9:31am
  
Introduction
  • 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
    • location
      • occurs most commonly in the diaphysis of long bones
      • femur and tibia are most common
  • Genetics
    • associated with ring chromosome 12
  • Prognosis
    • 20-35% chance of pulmonary metastasis
    • intermediate prognosis between parosteal and intramedullary osteosarcoma
    • 98% necrosis with 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
Presentation
  • Symptoms
    • pain is the most common presenting symptom
    • 25% present with pathologic fracture
  • Physical exam
    • regional swelling and tendernes 
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
 
  Location
Xray
Xray
CT
Bone scan
MRI
MRI
Case A tibia
Case B tibia
Case B proximal tibia

 

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Qbank (2 Questions)

TAG
(OBQ09.38) A 24-year-old man "twists" his knee in a softball accident. He denies any preceding complaints and is very active and healthy. Figures A through C include radiographs, MRI, and histology of the lesion. These findings are most consistent with what diagnosis? Topic Review Topic
FIGURES: A   B   C      

1. Parosteal osteosarcoma
2. Periosteal osteosarcoma
3. Osteochondroma
4. Dedifferentiated chondrosarcoma
5. Synovial Sarcoma

PREFERRED RESPONSE ▶
TAG
(OBQ06.47) A 47-year-old man presents with increasing left knee pain and swelling. There is no history of trauma and he is otherwise healthy. Representative radiograph, MRI, and histology are shown in Figures A through D. What is the most likely diagnosis? Topic Review Topic
FIGURES: A   B   C   D    

1. Chondrosarcoma
2. Ewing's sarcoma
3. Periosteal osteosarcoma
4. Giant cell tumor
5. Fibrous dysplasia

PREFERRED RESPONSE ▶




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