Updated: 5/4/2019

Giant Cell Tumor

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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/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/Histology B - parsons_moved.png
https://upload.orthobullets.com/topic/8046/images/screen_shot_2017-03-26_at_2.13.59_pm.jpg
Introduction
  • benign aggressive tumor typically found in the metaphysis of long bones 
  • Age & sex
    • more common in females (unlike most bone tumors which show male predominance)
    • ages 30-50 years 
  • 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 mobile spine, it usually occurs in the vertebral body 
    • distal radius is third most common location
    • phalanges of the hand is also a very common location
    • may arise in the apophysis (like chondroblastoma)
  • Malignancy
    • primary malignant giant cell tumor
      • metastatic to lung in 2-5%   
      • hand lesions have greater chance of metastasis
    • secondary malignant giant cell tumor 
      • occurs following radiation or multiple resections of giant cell tumor
Symptoms
  • Symptoms
    • pain referable to involved joint
  • Physical exam
    • palpable mass
    • decreased range of motion around affected joint
Imaging
  • Radiographs
    • 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
  • Chest radiograph or chest CT
    • pulmonary metatases occurs in 1-6%  
    • lung metastases are usually benign (histologically similar to primary bone tumor)
  • Bone scan
    • is very hot 
  • MRI
    • shows clear demarcation on T1 image between fatty marrow and tumor 
Histology
  • Characteristic cells  
    • Type I cell
      • mononucleur 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
      • denosumab acts on this pathway  
Treatment
  • Nonoperative
    • radiation alone
      • indications
        • only indicated for inoperable or multiply recurrent lesions
      • outcomes
        • leads to 15% malignant transformation
    • medical management
      • indications
        • medical therapy can be used to augment or replace surgical management depending on the specific clinical scenario
      • medications
        • bisphosphonates 
          • osteclast inhibitors which may decrease the size of the defect in giant cell tumors
        • denosumab 
          • monoclonal antibody against RANK-ligand
          • recent clinical trials suggest denosumab can decrease the size of the bone defect in giant cell tumor
            • 90% tumor necrosis
          • shows dramatic sclerosis and reconstitution of cortical bone after treatment  
  • Operative
    • extensive curettage and reconstruction (with adjuvant treatment)    
      • indications
        • lesions amenable to currettage
        • 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
      • 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 
        • can fill lesion with bone cement or autograft/allograft bone
      • outcomes
        • 10-30% recurrence with curettage alone verses 3% with adjuvant treatment (phenol, hydrogen peroxide, argon beam, etc)  
    • amputation
      • indications
        • hand lesions with cortical breakthrough who are not amendable to intercalary resection
Complications
  • Malignant transformation (<1% prevalence)
    • to high grade sarcoma (poor prognosis)
    • latency
      • 9 years for previous radiation treatment
      • 19 years for spontaneous transformation
  • Secondary ABC (≤14%)
    • differentiate from primary ABC because of enhancing soft-tissue component in GCT (not present in primary ABC)
  • Recurrence (15-25%)
    • lucency at bone-cement interface
      • diagnose with CT guided biopsy
  • Pathologic fracture
    • poorer outcome
  • Postoperative infection
    • increased risk with en bloc resection + endoprosthesis
 Differentials & Groups
  • 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

 
Epiphyseal lesion
   Treatment is USUALLY currettage and bone grafting(1)  

Giant Cell Tumor
   •    
Chondroblastoma
       
Aneurysmal Bone cyst      •    
Osteoblastoma      •    
Chondromyoid fibroma (CMF)      •    


       
ASSUMPTIONS: (1) assuming no impending fracture
 
IBank
  Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)
Case A knee -recurrence
 
 
 
Case B knee
 
 
 
 
 
Case C distal radius
 
 
 
 
 
Case D hand
 
 
 
 
 
Case B knee
 
 
 
 
Case B ankle
 
 
 
Case A humerus
 
 
 
 
 
(1) - histology does not always correspond to clinical case 
 

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Questions (38)
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(OBQ13.233) A 38-year-old man presents with progressive wrist swelling and pain for 8 months as seen in Figure A. He underwent imaging and biopsy as seen in Figures B and C. What is the most appropriate treatment for this patient? Review Topic

QID: 4868
FIGURES:
1

Neoadjuvant chemotherapy, radical resection, and chemotherapy

4%

(129/3059)

2

Neoadjuvant chemotherapy, radiotherapy, and wide resection

4%

(110/3059)

3

Radiotherapy and wide resection

4%

(115/3059)

4

Intralesional curettage and reconstruction with adjuvant treatments

87%

(2661/3059)

5

Amputation

1%

(25/3059)

L 2

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(SBQ12SP.76) A 55-year-old patient presents with lower lumbar back pain. A lateral radiograph of the L3 vertebral bone is shown in Figure A. Figure B and C are pre-operative biopsies. From the following options, what is the most likely diagnosis? Review Topic

QID: 3774
FIGURES:
1

Osteoblastoma

9%

(191/2235)

2

Osteoblastic osteosarcoma

9%

(198/2235)

3

Chondrosarcoma

18%

(403/2235)

4

Ewing's Sarcoma

1%

(33/2235)

5

Giant Cell Tumor

63%

(1398/2235)

L 3

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(SBQ12SP.105) A 50-year-old female presents with the radiograph seen in Figures A through D. Which of the following should be obtained to complete initial workup of this patient? Review Topic

QID: 3803
FIGURES:
1

MRI spine and brain

1%

(8/1291)

2

Obtain skeletal survey

7%

(87/1291)

3

SPEP and UPEP

2%

(25/1291)

4

Mammogram

1%

(15/1291)

5

CT chest

89%

(1151/1291)

L 1

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(OBQ10.204) A 29-year-old female complains of increasing severity back pain for the past 4 months. Radiograph, CT scan, T1 and T2 MRI, and biopsy specimen are shown in Figures A through E. What is the most likely diagnosis? Review Topic

QID: 3297
FIGURES:
1

Osteosarcoma

4%

(115/2593)

2

Ewing's sarcoma

2%

(42/2593)

3

Lymphoma

2%

(39/2593)

4

Giant cell tumor

59%

(1527/2593)

5

Chordoma

33%

(862/2593)

L 4

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(OBQ09.50) Which of the following radiographs demonstrates a tumor that would be best treated by curettage with a power burr and packing with cement? Review Topic

QID: 2863
FIGURES:
1

Figure A

5%

(97/1914)

2

Figure B

2%

(46/1914)

3

Figure C

1%

(12/1914)

4

Figure D

0%

(5/1914)

5

Figure E

91%

(1747/1914)

L 1

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(OBQ08.252) A 33-year-old female reports lateral knee pain and slight fullness. She underwent a bone scan which showed increased activity isolated to the proximal fibula. Radiographs and histology are shown in Figures A and B. What is the most likely diagnosis? Review Topic

QID: 638
FIGURES:
1

Parosteal osteosarcoma

1%

(22/1635)

2

Eosinophilic granuloma

13%

(217/1635)

3

Chondroblastoma

7%

(117/1635)

4

Giant cell tumor

77%

(1265/1635)

5

Multiple myeloma

1%

(9/1635)

L 2

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(OBQ07.186) Giant cell tumors of bone can be locally aggressive and result in significant bone destruction. Which of the following is responsible for this type of bone destruction? Review Topic

QID: 847
1

Multinuclear giant cells

16%

(193/1236)

2

Osteocytic stromal cells

3%

(37/1236)

3

Tumor cell activation of osteoclasts

74%

(920/1236)

4

Tumor cell inactivation of osteoblasts

1%

(14/1236)

5

Osteoprotegrin activation of osteoclasts

5%

(67/1236)

L 2

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(OBQ07.197) A 40-year-old female presents with dull pain in her knee that has been increasing in severity over the past 6 months. Figures A through D show representative radiographs, MRI, and biopsy section. What is the most appropriate treatment? Review Topic

QID: 858
FIGURES:
1

Observation

1%

(15/1139)

2

Bone marrow biopsy

2%

(23/1139)

3

Intra-lesional curettage with local adjuvant therapy

83%

(949/1139)

4

Neoadjuvant chemotherapy, surgical excision, and adjuvant chemotherapy

9%

(102/1139)

5

Neoadjuvant radiotherapy and surgical excision

4%

(43/1139)

L 1

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(OBQ06.73) A 41-year-old male presents with 6 months of wrist pain and swelling. He is otherwise healthy and denies trauma. Clincal photograph, radiographs, MRI, and histology are shown in Figures A through E. What is the most likely diagnosis? Review Topic

QID: 184
FIGURES:
1

Aneurysmal bone cyst

4%

(46/1250)

2

Giant cell tumor

89%

(1114/1250)

3

Telangectatic osteosarcoma

4%

(56/1250)

4

High-grade osteosarcoma

2%

(29/1250)

5

Osteomyelitis

0%

(2/1250)

L 1

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(OBQ06.114) A 21-year-old man presents with a lytic lesion in his distal femur. A chest radiograph reveals a lung nodule. Which of the following tumors most likely to metastasize to lung ? Review Topic

QID: 300
1

Non-ossifying fibroma

1%

(17/2186)

2

Giant cell tumor

94%

(2045/2186)

3

Aneurysmal bone cyst

1%

(22/2186)

4

Osteoid Osteoma

1%

(30/2186)

5

Hemangioma

3%

(65/2186)

L 1

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(OBQ05.203) All of the following locations are common sites for giant cell tumor of bone to occur EXCEPT? Review Topic

QID: 1089
1

Posterior elements of the spine

69%

(518/755)

2

Distal femur

2%

(13/755)

3

Sacrum

19%

(146/755)

4

Distal radius

6%

(48/755)

5

Proximal tibia

4%

(27/755)

L 3

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(OBQ05.240) Giant cell tumors of bone can occur in many different areas throughout the body. All of the following are common locations for giant cell tumors of bone EXCEPT? Review Topic

QID: 1126
1

Sacrum

15%

(80/523)

2

Distal radius

2%

(10/523)

3

Tibial diaphysis

69%

(363/523)

4

Distal femur

2%

(13/523)

5

Phalanges of the hand

10%

(54/523)

L 2

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(OBQ04.22) Figures A-E show skeletal distribution of common bone tumors. Which figure shows the most common sites of giant cell tumor distribution in bone? Review Topic

QID: 133
FIGURES:
1

Figure A

82%

(1155/1415)

2

Figure B

3%

(41/1415)

3

Figure C

4%

(56/1415)

4

Figure D

7%

(103/1415)

5

Figure E

4%

(54/1415)

L 2

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