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Updated: Jan 18 2024

Telangiectatic Osteosarcoma

Images
https://upload.orthobullets.com/topic/8017/images/Case A - prox hum - xray - parsons_moved.png
https://upload.orthobullets.com/topic/8017/images/Case C - distal femur - mri ax - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8017/images/Case C - distal femur - xray ap - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8017/images/Case C - distal femur - mri sag - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8017/images/Histology A - Parsons_moved.png
  • summary
    • Telangiectatic Osteosarcomas are rare, malignant, high-grade osteosarcomas that occur most commonly in the proximal humerus, the femur, and proximal tibia. Patients typically present between the ages of 5 and 25 with regional pain and swelling.
    • Diagnosis is made with biopsy showing high-grade sarcoma with mitotic figures is seen in intervening cellular areas and lakes of blood mixed with malignant cells. 
    • Treatment is usually neo-adjuvant chemotherapy, limb salvage surgical resection, followed by adjuvant chemotherapy.
  • Epidemiology 
    • Incidence
      • rare (4% of osteosarcomas)
    • Demographics
      • male > female
    • Anatomic location
      • proximal humerus, proximal femur, distal femur, proximal tibia
        • occur in same location as ABC
    • Risk factors
      • history of prior radiation
  • Etiology
    • Genetics
      • associated with the following mutations
        • tumor suppressor genes
          • Rb-1
          • p53
        • oncogenes
          • HER2/neu
          • c-myc
          • c-fos
  • Presentation
    • Symptoms
      • pain
        • similar in ABC in presentation (must differentiate)
      • 25% present with pathologic fracture
    • Physical exam
      • soft tissue swelling
      • local tenderness
  • Imaging
    • Radiographs
      • lytic, destructive, and expansile lesion; entire cortex may be compromised
    • Bone scan
      • shows increased uptake
    • MRI
      • indications
        • indicated in all cases to determine soft tissue involvement
      • findings
        • fluid-fluid levels
        • extensive edema in surrounding tissue
  • Studies
    • 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
    • Gross pathology
      • will show "bag of blood"
  • Differential
    • ABC 
      • diagnosis needs to be confirmed by an experienced musculoskeletal pathologist
      • differentiating ABC from telangietic osteosarcoma is difficult and critical
        • both have similar radiographic appearance
        • both have large blood filled spaces
        • both have similar location (proximal humerus, proximal femur, distal femur, proximal tibia)
      • ABC vs Telangiectatic Osteosarcoma 
      • Aneurysmal Bone Cyst
      • Telangiectatic Osteosarcoma 
      • Epidemiology
      • 75% of patient < 20 years.
      • Locations (proximal humerus, proximal femur, distal femur, proximal tibia)
      • Locations (proximal humerus, proximal femur, distal femur, proximal tibia)
      • Radiographs
      • Expansive, eccentric and lytic lesion with bony septae and cortical thinning
      • Expansive, eccentric and lytic lesion with bony septae and possible cortical disruption
      • MRI
      • Clear fluid levels
      • May extend into soft tissue.
      • Fluid levels less defined
      • Edema in surrounding tissue
      • Histology
      • Blood-filled spaces, spindle cells, benign giant cells, no evidence of malignant cells
      • Lakes of blood mixed with malignant cells with mitotic bodies
  • Treatment
    • Operative
      • neoadjuvant chemotherapy, limb salvage resection, followed by adjuvant chemotherapy
        • 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
          • risk of recurrence is decreased with good cellular response/wide surgical margins
  • Complications
    • Local recurrence
      • uncommon (~5%)
      • associated with poor prognosis
      • increased risk of recurrence in patients with displaced, pathologic fracture
  • Prognosis
    • Prognostic variables
      • poor
        • advanced tumor stage is most important indicator
        • increased lactate dehydrogenase (LDH) and alkaline phosphatase (ALP)
        • expression of multi-drug resistance (MDR) gene
        • pathologic fractures increased risk of recurrence
      • favorable
        • 98% necrosis with chemo is good prognostic sign
    • 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%
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