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https://upload.orthobullets.com/topic/8046/images/Case D - distal radius - bone scan - parsons_moved.gif
https://upload.orthobullets.com/topic/8046/images/Case I - knee - MRI T1 - parsons_moved.png
https://upload.orthobullets.com/topic/8046/images/Case J - ankle - xray - parsons_moved.jpg
https://upload.orthobullets.com/topic/8046/images/Case J - ankle - T1- parsons_moved.jpg
https://upload.orthobullets.com/topic/8046/images/Histology E_moved.jpg
https://upload.orthobullets.com/topic/8046/images/Histology B - parsons_moved.png
https://upload.orthobullets.com/topic/8046/images/screen_shot_2017-03-26_at_2.13.59_pm.jpg
  • Summary
    • Giant Cell Tumors are benign, aggressive tumors typically found in the epiphysis of long bones, most commonly at the distal femur and proximal tibia. 
    • Patients typically present between ages 30 and 50 with insidious onset of pain of the involved extremity with activity, at night, or at rest.
    • Diagnosis is made with a biopsy showing mononuclear stromal cells that resemble interstitial fibroblasts with numerous giant cells dispersed throughout. 
    • Treatment is generally curettage, adjuvant treatment, and reconstruction as necessary depending on the location of the lesion.
  • Epidemiology
    • Incidence
      • 1.7 per million people
    • Demographics
      • more common in females (unlike most bone tumors which show male predominance)
      • ages 30-50 years
    • Anatomic location
      • distal femur > proximal tibia > distal radius > sacral ala
        • 50% occur around knee (distal femur or proximal tibia)
        • 10% in sacrum and vertebrae (sacral ala is most common site in axial skeleton)
          • in the spine it usually occurs in the vertebral body
      • phalanges of the hand is also a very common location
      • may arise in the apophysis (like chondroblastoma)
  • Etiology
    • Genetics
      • mutations
        • metastatic lesions often noted to have altertions in c-myc oncogene or p53
    • Associated conditions
      • malignancy
        • primary malignant giant cell tumor
          • metastatic to lung in 2-4% of cases
          • wrist and hand lesions have greater chance of metastasis
        • secondary malignant giant cell tumor
          • occurs following radiation or multiple resections of giant cell tumor
  • Presentation
    • Symptoms
      • pain
        • insidious onset of pain of the involved extremity with activity, at night, or at rest
        • pain referable to involved joint
        • night pain (result of tumor expansion)
        • difficulty ambulating
      • swelling
    • Physical exam
      • inspection & palpation
        • palpable mass
        • tenderness over mass
        • soft tissue swelling
          • if peri-articular lesion, joint effusion can be present
      • motion
        • decreased range of motion around affected joint
      • gait
        • antalgic
  • Imaging
    • Radiographs
      • recommended views
        • extremity involved
        • chest x-ray
          • to evaluate for lung metastasis
      • findings
        • eccentric lytic epiphyseal/metaphyseal lesion that often extends into the distal epiphysis and borders subchondral bone
        • "neo-cortex" is characteristic of benign aggressive lesions, and not unique to GCT
    • CT
      • recommend views
        • chest CT
          • to evaluate for pulmonary metatases occurs in 1-6%
      • findings
        • lung metastases are usually benign (histologically similar to primary bone tumor)
    • MRI
      • indications
        • to evalute for extent of lesion
      • findings
        • tumor blushing
        • cystic degeneration
        • shows clear demarcation on T1 image between fatty marrow and tumor
          • dark on T1, bright on T2 and avid on gadolinium enhanced
    • Bone scan
      • findings
        • Can be variable
  • Studies
    • Histology
      • characteristic cells
        • type I cell
          • mononuclear stromal cell that resembles interstitial fibroblasts
          • this is the neoplastic/tumor cell
          • has features of mesenchymal stem cells
        • type II cell
          • from monocyte/macrophage family recruited from peripheral blood
          • precursors of giant cells
        • type III cell
          • numerous giant cells are the hallmark of this lesion
          • nuclei
            • nuclei of giant cell appears same as stromal cells
            • multiple nuclei (up to 50 per cell)
          • similar characteristics as osteoclasts and resorb bone
            • have same enzymes (tartrate resistant acid phosphatase, carbonic anhydrase II, cathepsin K, vacuolar ATPase)
        • secondary ABC degeneration is not uncommon
      • molecular biology
        • type II and III cells have IGF-I and IGF-II activity
        • 80% of patients with GCT have telomeric associations (tas) abnormality in half the cells
        • RANK pathway is important
  • Differential
    • Brown tumor of hyperparathyroidism
      • can look like GCT on radiographs except it occurs as multiple lesions and associated with serum calcium level abnormalities
    • Chondroblastoma
      • epiphyseal location
      • may also demonstrate ABC formation
      • has extensive surrounding soft tissue and marrow edema
      • may have sclerotic margin and central calcification of chondroid matrix "ring and arcs" pattern
    • Osteosarcoma
      • telangiectatic OS
      • giant cell-rich OS
      • fibroblastic OS
    • Chordoma (mimics GCT sacrum)
      • occurs in midline
      • Differential of Giant Cell tum
      • Epiphyseal lesion
      • Treatment is USUALLY curettage and bone grafting
      • Giant Cell Tumor
      • o
      • o
      • Chondroblastoma
      • o
      • o
      • Aneurysmal Bone cyst
      • o
      • Osteoblastoma
      • o
      • Chondromyoid fibroma (CMF)
      • o
  • Treatment
    • Nonoperative
      • radiation therapy
        • indications
          • only indicated for inoperable or multiply recurrent lesions
            • sacral lesions and large vertebral body lesions that are not ammendable to surgery
        • outcomes
          • leads to 15% malignant transformation
      • medical management (denosumab, bisphosphanates)
        • indications
          • stabilize lesions
          • augment or replace surgical management depending on the specific clinical scenario
        • outcomes
          • excellent response has been seen with denosumab (85-90% destruction of giant cells)
          • post-surgical treatment with diphosphonate has shown to lower recurrence rates by 25-30%
    • Operative
      • extensive curettage, adjuvant treatment and reconstruction
        • indications
          • lesions amenable to currettage
          • majority of lower extremity lesions
          • hand lesion treatment is most controversial
        • outcomes
          • 20-40% recurrence with curettage and bone grafting alone versus 3-10% with addition of adjuvant treatment (phenol, hydrogen peroxide, cryo, argon beam, high-speed burr)
      • complete resection and reconstruction
        • indications
          • when currettage not possible due to structural compromise
            • with extensive involvement of vertebral body complete en bloc spondylectomy may be required
        • outcomes
          • the 10 year disease-free survival rates after total en bloc spondylectomy for GCT is reported to be 100% in some studies.
      • amputation
        • indications
          • hand lesions with cortical breakthrough who are not amendable to intercalary resection
        • outcomes
          • has the lowest incidence of recurrence
  • Techniques
    • Radiation therapy
      • technique
        • external beam radiation
    • Medical management
      • technique
        • bisphosphonates
          • osteclast inhibitors which may decrease the size of the defect in giant cell tumors and help prevent post-surgical recurrence
        • denosumab
          • monoclonal antibody against RANK-ligand
          • recent clinical trials suggest denosumab can decrease the size of the bone defect in giant cell tumor
            • 85-90% tumor necrosis
          • shows dramatic sclerosis and reconstitution of cortical bone after treatment
        • complications
          • bisphosphonates
            • esophagitis, gastritis, long term use can lead to atypical subtrochanteric femur fractures
          • denosumb
            • may cause nasopharyngitis, arthralgias
            • contraindications
              • severe hypocalcemia
    • Extensive curettage, adjuvant treatment and reconstruction
      • extensive curettage
        • technique
          • challenge of treatment is to remove lesion while preserving joint and providing support to subchondral joint
          • extensive exterioration (removal of a large cortical window over the lesion) is required
          • hand curettage is the most controversial
            • if no cortical breakthrough treat with curettage and cementing 
            • if significant cortical breakthrough consider intercalary resection (with free fibular graft) vs. amputation
        • adjuvant treatment
          • technique
            • phenol, liquid nitrogen, hydrogen peroxide, argon beam, high-speed burr
              • can be effectively used in isolation or combination to reduce recurrence rates
              • local recurrence rates with supplementation of ethanol and phenol are identical
              • high-speed burr without any other adjuvant has a recurrence rate of 12%
            • complications
              • liquid nitrogen (aka cryotherapy) has been associated with an increased incidence of pathologic fracture and vascular injury
        • reconstruction
          • technique
            • fill lesion with bone cement or bone graft and supplement with internal fixation
            • structural allograft, endoprosthetic implants or combinations of two for large lesions or in setting of extensive bone destruction
    • Complete resection and reconstruction
      • approach
        • based on location in the spine (lumbar, thoracic, cervical)
        • anterior, posterior, or combined
          • anterior approach in the lumbar spine may be direct lateral, oblique lateral or transperitoneal depending on specific level
        • technique
          • can be single or 2-staged procedure
          • 1st stage would involve total vertebral spondylectomy, adjuvant treatment with the placement of a reconstruction expandable cage and anterior plating
            • alternatively can use bone cement in lieu of a structural cage
            • may require nerve root resection based on spinal level and involvement of nerve roots within tumor
          • 2nd stage would involve bilateral laminectomy and excision of remaining soft tissues following by posterior instrumentation
    • Amputation
      • technique
        • resection of phalangeal or metacarpal lesions with ray transfer or allograft reconstruction
  • Complications
    • Malignant transformation to high grade sarcoma
      • incidence
        • very rare (<1% prevalence)
        • latency
          • 9 years from previous radiation treatment
          • 19 years from spontaneous transformation
      • treatment
        • surgical resection of metastatic lesions
        • interferon treatment
        • radiation
    • Secondary ABC
      • incidence
        • between 10-14%
        • differentiate from primary ABC because of enhancing soft-tissue component in GCT (not present in primary ABC)
    • Recurrence
      • incidence
        • local recurrence occurs in 20% cases
        • diagnose with CT guided biopsy
      • risk factors
        • not using adjuvant treatment during surgery
    • Pathologic fracture
      • risk factors
        • peri-articular lesions
        • using crytherapy as an adjuvant
  • Prognosis
    • Risk of malignancy < 5 %
      • metastatic GCT has a 5 year 76% disease-free survival rate and a 17% mortality rate.
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