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Telangiectatic Osteosarcoma

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Topic updated on 03/13/13 4:05pm
  
Introduction
  • A malignant osteogenic tumor
    • similar to classic osteosarcoma in epidemiology and genetics
    • similar in ABC in presentation (must differentiate)
  • Epidemiology (similar to classic osteosarcoma)
    • incidence
      • rare (4% of osteosarcomas)
    • demographics
      • male > female
    • location
      • proximal humerus, proximal femur, distal femur, proximal tibia
        • occur in same location as ABC
  • Genetics 
    • Rb-1 mutations
    • Inc incidence in those with mutations in p53, hx of prior radiation
    • Over-expression of oncogenes--HER2/neu, c-myc, c-fos 
  • Prognosis
    • prognostic variable
      • tumor stage is most important indicator
      • increased lactate dehydrogenase (LDH) and alkaline phosphatase (ALP) are poor prognostic indicators
      • expression of multi-drug resistance (MDR) gene portends very poor prognosis
    • survival
      • more chemosensitive but same survival as intramedullary osteosarcoma
      • 5 year survival with tumor localized to an extremity is ~70%
      • 5 year survival with metastases is ~20%
Presentation
  • Symptoms
    • 25% present with pathologic fracture
Imaging
  • Radiographs
    • lytic, destructive, and expansile lesion; entire cortex may be compromised
      • Differential dx: ABC
  • Bone scan
    • Increased uptake
  • MRI
    • fluid-fluid levels 
    • extensive edema in surrounding tissue
Studies
  • Gross pathology
    • "bag of blood"
  • Histology
    • characterized by
      • high grade sarcoma with mitotic figures is seen in intervening cellular areas
      • lakes of blood mixed with malignant cells (not in ABC) 
      • not as much osteoid as intramedullary osteosarcoma 
      • histology is critical to differentiate from ABC
Differential
  • ABC
    • similar radiographic appearance
    • both have large blood filled spaces
    • similar location
      • proximal humerus, proximal femur, distal femur, proximal tibia
Treatment
  • Operative  
    • multi-agent  (neoadjuvant) chemotherapy and limb salvage resection 
      • indications
        • standard of care in most patients 
      • surgical resection
        • trend towards limb salvage whenever possible
          • can be performed in close to 90% of cases
      • chemotherapy
        • preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 6-12 months after surgical resection
          • doxorubicin/cisplatin/methotrexate/ifosamide
        • 98% necrosis with chemo is good prognostic sign
        • expression of multi-drug resistance (MDR) gene portends very poor prognosis
          • cells can pump chemo out of cell
          • present in 25% of primary lesions and 50% of metastatic lesions
      • outcomes
        • increased risk of recurrence in patients with displaced, pathologic fracture
        • risk of recurrence is decreased with good cellular response/wide surgical margins
Complications
  • Local recurrence
    • uncommon (~5%) 
    • associated with poor prognosis
  Location
Xray
Xray
CT
Bone scan
MRI
MRI
Case A prox. humerus
 
Case B distal femur
Case C distal femur

 

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Qbank (1 Questions)

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(OBQ10.124) a 21-year-old male has 6 months of increasing knee pain and has recently noticed a mass at his knee. Radiographs are shown in Figures A and B. A biopsy specimen of the proximal tibia mass is shown in Figure C. What is the most appropriate first step in management? Topic Review Topic
FIGURES: A   B   C      

1. Neoadjuvant chemotherapy
2. Wide surgical excision
3. Intralesional curettage and bone grafting
4. Radiation therapy
5. Hip disarticulation

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