Updated: 6/22/2021

Giant Cell Tumor

0%
Topic
Review Topic
0
0
N/A
N/A
Questions
35
0
0
0%
0%
Evidence
42
0
0
0%
0%
Videos / Pods
1
0%
0%
Cases
7
Topic
Images
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/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 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
      Chondroblastoma
      Aneurysmal Bone cyst
      Osteoblastoma
      Chondromyoid fibroma (CMF)
  • 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.
Flashcards (0)
Cards
1 of 0
Questions (35)
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(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?

QID: 4868
FIGURES:
1

Neoadjuvant chemotherapy, radical resection, and chemotherapy

4%

(145/3534)

2

Neoadjuvant chemotherapy, radiotherapy, and wide resection

3%

(123/3534)

3

Radiotherapy and wide resection

4%

(133/3534)

4

Intralesional curettage and reconstruction with adjuvant treatments

87%

(3082/3534)

5

Amputation

1%

(29/3534)

L 2 B

Select Answer to see Preferred Response

(SBQ12SP.106) A 33-year-old female presents to the orthopedic oncologist with the radiographs shown in Figure A. She subsequently undergoes extensive curettage with local phenol and cementation as adjuvants. Which of the following histologic slides is consistent with this patient’s pathology?

QID: 3804
FIGURES:
1

Figure B

3%

(42/1431)

2

Figure C

5%

(76/1431)

3

Figure D

83%

(1185/1431)

4

Figure E

6%

(82/1431)

5

Figure F

2%

(35/1431)

L 2 C

Select Answer to see Preferred Response

(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?

QID: 3803
FIGURES:
1

MRI spine and brain

1%

(13/1767)

2

Obtain skeletal survey

7%

(125/1767)

3

SPEP and UPEP

2%

(41/1767)

4

Mammogram

1%

(21/1767)

5

CT chest

88%

(1557/1767)

L 1 B

Select Answer to see Preferred Response

(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?

QID: 3774
FIGURES:
1

Osteoblastoma

8%

(239/3137)

2

Osteoblastic osteosarcoma

8%

(263/3137)

3

Chondrosarcoma

17%

(540/3137)

4

Ewing's Sarcoma

2%

(50/3137)

5

Giant Cell Tumor

65%

(2027/3137)

L 3 C

Select Answer to see Preferred Response

Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(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?

QID: 3297
FIGURES:
1

Osteosarcoma

5%

(134/2902)

2

Ewing's sarcoma

2%

(48/2902)

3

Lymphoma

2%

(52/2902)

4

Giant cell tumor

58%

(1691/2902)

5

Chordoma

33%

(966/2902)

L 4 B

Select Answer to see Preferred Response

(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?

QID: 2863
FIGURES:
1

Figure A

5%

(129/2396)

2

Figure B

3%

(61/2396)

3

Figure C

1%

(22/2396)

4

Figure D

0%

(8/2396)

5

Figure E

91%

(2169/2396)

L 1 B

Select Answer to see Preferred Response

Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(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?

QID: 638
FIGURES:
1

Parosteal osteosarcoma

1%

(24/1920)

2

Eosinophilic granuloma

13%

(248/1920)

3

Chondroblastoma

7%

(134/1920)

4

Giant cell tumor

78%

(1497/1920)

5

Multiple myeloma

0%

(9/1920)

L 2 A

Select Answer to see Preferred Response

Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(OBQ07.186) A 34-year-old female presents to the office with complaints of right knee pain for the past 6 months, her radiograph is shown in Figure F. Which of the following histological slides is consistent with the patient's pathology?

QID: 847
FIGURES:
1

Figure A

15%

(248/1620)

2

Figure B

3%

(49/1620)

3

Figure C

74%

(1206/1620)

4

Figure D

2%

(29/1620)

5

Figure E

5%

(82/1620)

L 2 B

Select Answer to see Preferred Response

(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?

QID: 858
FIGURES:
1

Observation

1%

(16/1509)

2

Bone marrow biopsy

3%

(40/1509)

3

Intra-lesional curettage with local adjuvant therapy

82%

(1240/1509)

4

Neoadjuvant chemotherapy, surgical excision, and adjuvant chemotherapy

10%

(145/1509)

5

Neoadjuvant radiotherapy and surgical excision

4%

(60/1509)

L 1 C

Select Answer to see Preferred Response

(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?

QID: 184
FIGURES:
1

Aneurysmal bone cyst

4%

(65/1616)

2

Giant cell tumor

88%

(1427/1616)

3

Telangectatic osteosarcoma

4%

(70/1616)

4

High-grade osteosarcoma

3%

(42/1616)

5

Osteomyelitis

0%

(3/1616)

L 1 D

Select Answer to see Preferred Response

(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 ?

QID: 300
1

Non-ossifying fibroma

1%

(20/2525)

2

Giant cell tumor

94%

(2366/2525)

3

Aneurysmal bone cyst

1%

(23/2525)

4

Osteoid Osteoma

1%

(36/2525)

5

Hemangioma

3%

(73/2525)

L 1 B

Select Answer to see Preferred Response

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

QID: 1089
1

Posterior elements of the spine

69%

(723/1044)

2

Distal femur

2%

(16/1044)

3

Sacrum

20%

(214/1044)

4

Distal radius

5%

(52/1044)

5

Proximal tibia

3%

(36/1044)

L 3 C

Select Answer to see Preferred Response

(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?

QID: 1126
1

Sacrum

16%

(123/755)

2

Distal radius

2%

(12/755)

3

Tibial diaphysis

70%

(530/755)

4

Distal femur

2%

(17/755)

5

Phalanges of the hand

9%

(70/755)

L 2 B

Select Answer to see Preferred Response

(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?

QID: 133
FIGURES:
1

Figure A

81%

(1486/1826)

2

Figure B

3%

(55/1826)

3

Figure C

4%

(82/1826)

4

Figure D

7%

(130/1826)

5

Figure E

4%

(64/1826)

L 2 B

Select Answer to see Preferred Response

Evidence (42)
VIDEOS & PODCASTS (2)
CASES (7)
EXPERT COMMENTS (27)
Private Note