Updated: 5/3/2020

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
  • Overview
    • A Giant Cell Tumor (GCT) is a benign aggressive tumor typically found in the metaphysis of long bones, often around the kneein young adults
      • treatment is generally curretage, adjuvant treatment, and reconstruction as necessary depending on location of 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 
    • 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)
  • 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
  • Prognosis
    • metastatic GCT has a 5 year 76% disease-free survival rate and a 17% mortality rate.
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
      • is very hot  
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 
        • denosumab acts on this pathway  
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
 
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
 
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 curretage, adjuvant treatment and reconstruction
    • extensive curretage
      • 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 curretage is the most controversal
          • 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 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 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
 Differentials & Groups
 
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 (34)
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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 | Tested Concept

QID: 4868
FIGURES:
1

Neoadjuvant chemotherapy, radical resection, and chemotherapy

4%

(134/3282)

2

Neoadjuvant chemotherapy, radiotherapy, and wide resection

4%

(117/3282)

3

Radiotherapy and wide resection

4%

(124/3282)

4

Intralesional curettage and reconstruction with adjuvant treatments

87%

(2861/3282)

5

Amputation

1%

(27/3282)

L 2 B

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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 | Tested Concept

QID: 3774
FIGURES:
1

Osteoblastoma

8%

(218/2656)

2

Osteoblastic osteosarcoma

9%

(233/2656)

3

Chondrosarcoma

18%

(478/2656)

4

Ewing's Sarcoma

2%

(41/2656)

5

Giant Cell Tumor

63%

(1672/2656)

L 3 B

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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 | Tested Concept

QID: 3803
FIGURES:
1

MRI spine and brain

1%

(9/1419)

2

Obtain skeletal survey

7%

(99/1419)

3

SPEP and UPEP

2%

(31/1419)

4

Mammogram

1%

(16/1419)

5

CT chest

89%

(1258/1419)

L 1 B

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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 | Tested Concept

QID: 3297
FIGURES:
1

Osteosarcoma

4%

(116/2661)

2

Ewing's sarcoma

2%

(44/2661)

3

Lymphoma

2%

(41/2661)

4

Giant cell tumor

58%

(1555/2661)

5

Chordoma

34%

(895/2661)

L 4 B

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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 | Tested Concept

QID: 2863
FIGURES:
1

Figure A

5%

(109/2134)

2

Figure B

2%

(49/2134)

3

Figure C

1%

(14/2134)

4

Figure D

0%

(7/2134)

5

Figure E

91%

(1947/2134)

L 1 B

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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 | Tested Concept

QID: 638
FIGURES:
1

Parosteal osteosarcoma

1%

(22/1740)

2

Eosinophilic granuloma

13%

(224/1740)

3

Chondroblastoma

7%

(122/1740)

4

Giant cell tumor

78%

(1357/1740)

5

Multiple myeloma

1%

(9/1740)

L 2 A

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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 | Tested Concept

QID: 847
1

Multinuclear giant cells

17%

(226/1367)

2

Osteocytic stromal cells

3%

(41/1367)

3

Tumor cell activation of osteoclasts

74%

(1006/1367)

4

Tumor cell inactivation of osteoblasts

1%

(16/1367)

5

Osteoprotegrin activation of osteoclasts

5%

(73/1367)

L 2 B

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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 | Tested Concept

QID: 858
FIGURES:
1

Observation

1%

(15/1206)

2

Bone marrow biopsy

2%

(25/1206)

3

Intra-lesional curettage with local adjuvant therapy

84%

(1008/1206)

4

Neoadjuvant chemotherapy, surgical excision, and adjuvant chemotherapy

9%

(108/1206)

5

Neoadjuvant radiotherapy and surgical excision

4%

(43/1206)

L 1 B

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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 | Tested Concept

QID: 184
FIGURES:
1

Aneurysmal bone cyst

4%

(52/1357)

2

Giant cell tumor

89%

(1202/1357)

3

Telangectatic osteosarcoma

4%

(61/1357)

4

High-grade osteosarcoma

2%

(33/1357)

5

Osteomyelitis

0%

(2/1357)

L 1 C

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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 | Tested Concept

QID: 300
1

Non-ossifying fibroma

1%

(18/2347)

2

Giant cell tumor

94%

(2198/2347)

3

Aneurysmal bone cyst

1%

(22/2347)

4

Osteoid Osteoma

1%

(33/2347)

5

Hemangioma

3%

(69/2347)

L 1 B

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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 | Tested Concept

QID: 1089
1

Posterior elements of the spine

69%

(561/810)

2

Distal femur

2%

(13/810)

3

Sacrum

19%

(157/810)

4

Distal radius

6%

(49/810)

5

Proximal tibia

3%

(27/810)

L 3 C

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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 | Tested Concept

QID: 1126
1

Sacrum

16%

(90/566)

2

Distal radius

2%

(10/566)

3

Tibial diaphysis

69%

(390/566)

4

Distal femur

2%

(14/566)

5

Phalanges of the hand

10%

(59/566)

L 2 B

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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 | Tested Concept

QID: 133
FIGURES:
1

Figure A

81%

(1249/1538)

2

Figure B

3%

(44/1538)

3

Figure C

5%

(74/1538)

4

Figure D

7%

(109/1538)

5

Figure E

4%

(55/1538)

L 2 B

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