Updated: 2/16/2020

Telangiectatic Osteosarcoma

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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
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
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
    • risk factors
      •  history of prior radiation
  • Genetics
    • associated with the following mutations
      • tumor suppressor genes
        • Rb-1
        • p53
      • oncogenes
        • HER2/neu
        • c-myc
        • c-fos 
  • 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%
Presentation
  • Symptoms
    • pain
    • 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
     ABC
    • 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)
    • diagnosis needs to be confirmed by an experienced musculoskeletal pathologist
ABC vs Telangietic Osteosarcoma
  Aneurysmal Bone Cyst
Telangietic 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 • Expansive, eccentric and lytic lesion with bony septae
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  
    • 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
        • 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
Ibank
  Location
Xray
Xray
CT
Bone scan
MRI
MRI
Case A prox. humerus
 
Case B distal femur
Case C distal femur
 

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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? Review Topic | Tested Concept

QID: 3218
FIGURES:
1

Neoadjuvant chemotherapy

38%

(911/2404)

2

Wide surgical excision

11%

(270/2404)

3

Intralesional curettage and bone grafting

49%

(1189/2404)

4

Radiation therapy

1%

(28/2404)

5

Hip disarticulation

0%

(1/2404)

L 5 C

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