Updated: 10/23/2017

Periosteal Osteosarcoma

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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
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
    • germ-line mutation of p53 found in 15-20% of cases 
  • 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 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
 
  Location
Xray
Xray
CT
Bone scan
MRI
MRI
Case A tibia
Case B tibia
Case B proximal tibia
 

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Questions (12)

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(OBQ13.229) A 12-year-old boy was diagnosed with Li–Fraumeni syndrome. The origin of his syndrome is related to a germ-line mutation of the tumor suppressor p53 gene. What is most common primary bone tumor associated with this gene mutation? Review Topic

QID: 4864
1

Chondrosarcoma

4%

(129/3013)

2

Osteosarcoma

77%

(2312/3013)

3

Spindle cell sarcoma of bone

4%

(120/3013)

4

Ewing's sarcoma

13%

(391/3013)

5

Multiple myeloma

1%

(44/3013)

L 2

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(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 and B include radiographs and histology of the lesion. These findings are most consistent with what diagnosis? Review Topic

QID: 2851
FIGURES:
1

Parosteal osteosarcoma

21%

(516/2425)

2

Periosteal osteosarcoma

68%

(1643/2425)

3

Osteochondroma

2%

(38/2425)

4

Dedifferentiated chondrosarcoma

7%

(166/2425)

5

Synovial Sarcoma

2%

(57/2425)

L 3

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(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? Review Topic

QID: 158
FIGURES:
1

Chondrosarcoma

9%

(232/2495)

2

Ewing's sarcoma

1%

(35/2495)

3

Periosteal osteosarcoma

87%

(2178/2495)

4

Giant cell tumor

1%

(24/2495)

5

Fibrous dysplasia

1%

(16/2495)

L 1

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