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20110922

Giant cell tumour



  
Introduction
  • benign but aggressive tumor of unknown origin that is usually found in the epiphysis of long bones 
  • Age
    • more common in females (unlike most bone tumors)
    • ages 20-40 years (80% of giant cell tumors occur in patients older than age 20 years, with the peak incidence in the third decade of life)
      • patient age/gender on previous OITE questions: 17F, 21M, 27F, 34F, 36F, 40M 
  • Location
    • 50% around knee (distal femur or proximal tibia), most common location 
    • 10% in sacrum and vertebrae (sacrum is most common site in axial skeleton) 
    • distal radius is third most common location
    • phalanges of the hand is also a very common location
  • Malignant potential as
    • primary malignant giant cell tumor
      • metastasizes to lung in 2% 
      • hand lesions have greater chance of metastasis
    • secondary malignant giant cell tumor
      • occurs following radiation or multiple resections of giant cell tumor
  • Differential
    • If multiple lesions (~1%) than rule out hyperparathyroidism

Symptoms
  • Presentation  
    • pain referable to involved joint
Imaging
  • Radiographs
    • lytic and destructive metaphyseal lesion that often extends into the epiphysis and often borders subchondral bone 
      • may have cortical thinning with breakthrough
  • Bone scan is very hot 
  • MRI shows clear demarcation on T1 image between fatty marrow and tumor 
Histology
  • Characteristic findings
    • basic proliferating cell type is the mononucleur spindle-shaped stromal cell 
    • hallmark Giant cells are numerous 
      • nucleus of giant cell appears same as spindle-shaped stromal cells
Treatment
  • Operative
    • extensive curettage with chemical cauterization (phenol), bone grafting and cementing 
      • challenge of treatment is to remove lesion while preserving joint and providing buttress support to subchondral joint
      • aggressive exterioration of overlying cortex is required 
      • 10-30% recurrence with curettage alone verses 3% with cementation
    • hand lesion treatment is controversial
      • if no cortical breakthrough treat with curettage and cementing
      • if significant cortical breakthrough consider intercalary resection (with free fibular graft) vs. amputation
    • radiation alone for inoperable lesions only
      • leads to 15% malignant transformation

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