MCID: BNG036
MIFTS: 48

Bone Giant Cell Tumor

Categories: Bone diseases, Cancer diseases, Rare diseases

Aliases & Classifications for Bone Giant Cell Tumor

MalaCards integrated aliases for Bone Giant Cell Tumor:

Name: Bone Giant Cell Tumor 12 15 17
Giant Cell Tumor of Bone 12 53 59 37 44 72
Osteoclastoma 12 53 59 55
Gct of Bone 53 59
Giant Cell Neoplasm of Bone 12
Giant Cell Myeloma 12

Classifications:

Orphanet: 59  
Rare bone diseases


External Ids:

Disease Ontology 12 DOID:4305
KEGG 37 H01470
MeSH 44 D018212
NCIt 50 C121932
SNOMED-CT 68 57500000
ICD10 via Orphanet 34 D48.0
UMLS via Orphanet 73 C0206638
Orphanet 59 ORPHA363976
UMLS 72 C0206638

Summaries for Bone Giant Cell Tumor

KEGG : 37
Giant-cell tumor of bone (GCTB) is a rare osteolytic tumor of the bone. Although classified as a benign tumor, GCTB is characterized by local aggressiveness and risk of local recurrence. Its name is derived from the numerous multinucleated giant cells found within the tumor, which are principally responsible for the extensive bone resorption that is characteristic of GCTB. However, the neoplastic components of GCTB are the spindle-like stromal cells, which produce RANKL that recruits monocytic osteoclast precursors from blood to the tumor, and stimulates differentiation into multinucleated giant cells. Recently, denosumab (RANKL inhibitor) has become a new treatment option for locally advanced GCTB.

MalaCards based summary : Bone Giant Cell Tumor, also known as giant cell tumor of bone, is related to giant cell reparative granuloma and giant cell tumor, and has symptoms including pain An important gene associated with Bone Giant Cell Tumor is ACP5 (Acid Phosphatase 5, Tartrate Resistant), and among its related pathways/superpathways are Degradation of the extracellular matrix and RANK Signaling in Osteoclasts. The drugs Denosumab and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include bone, monocytes and lung, and related phenotypes are immune system and craniofacial

Disease Ontology : 12 A giant cell tumor and bone cancer and bone tissue neoplasm that results in the presence of multinucleated giant cells (osteoclast-like cell).

Wikipedia : 75 Giant-cell tumor of the bone, (GCTOB) also called osteoclastoma, is a relatively uncommon tumor of the... more...

Related Diseases for Bone Giant Cell Tumor

Diseases related to Bone Giant Cell Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 159)
# Related Disease Score Top Affiliating Genes
1 giant cell reparative granuloma 30.7 CALCR CALCA
2 giant cell tumor 30.7 TNFSF11 SERPINA3 CTSK CALCA
3 multicentric carpotarsal osteolysis syndrome 30.2 TNFSF11 CTSK
4 bone inflammation disease 30.2 TNFSF11 CTSK ACP5
5 villonodular synovitis 30.1 TNFSF11 ACP5
6 bone resorption disease 30.0 TNFSF11 SPP1 CTSK CALCA ACP5
7 osteomyelitis 29.9 TNFSF11 CTSK ACP5
8 aneurysmal bone cysts 29.8 TNFSF11 CALCA
9 pigmented villonodular synovitis 29.6 TNFSF11 CALCR ACP5
10 endosteal hyperostosis, autosomal dominant 29.4 TNFSF11 CTSK
11 bone disease 28.8 TNFSF11 SPP1 CTSK CALCA ACP5
12 bone giant cell sarcoma 11.4
13 paget disease of bone 6 11.3
14 osteogenic sarcoma 10.7
15 malignant giant cell tumor 10.6
16 chondroblastoma 10.4
17 tendinitis 10.4 SPP1 ACP5
18 histiocytoma 10.3
19 fibrous histiocytoma 10.3
20 undifferentiated pleomorphic sarcoma 10.3
21 secondary hyperparathyroidism of renal origin 10.3 CALCA ACP5
22 malignant fibroxanthoma 10.3
23 hypophosphatasia, adult 10.3 SPP1 CALCA
24 epulis 10.3
25 fibroma 10.3
26 hypercementosis 10.2 SPP1 CTSK ACP5
27 calcific tendinitis 10.2 SPP1 CTSK
28 osteonecrosis 10.2
29 ossifying fibroma 10.2
30 fibrosarcoma 10.2
31 osteonecrosis of the jaw 10.2
32 mammary paget's disease 10.2
33 chondrosarcoma 10.1
34 sarcoma 10.1
35 spindle cell sarcoma 10.1
36 rapidly involuting congenital hemangioma 10.1
37 periapical periodontitis 10.1 TNFSF11 ACP5
38 paget disease of bone 5, juvenile-onset 10.1 TNFSF11 CALCA
39 paget's disease of bone 10.1
40 hairy cell leukemia 10.1
41 osteoblastoma 10.1
42 hyperparathyroidism 10.1
43 mesenchymal cell neoplasm 10.1
44 48,xyyy 10.1
45 ring chromosome 10.1
46 ischemic bone disease 10.0 TNFSF11 ACP5
47 fibrous dysplasia 10.0
48 primary hyperparathyroidism 10.0
49 liposarcoma 10.0
50 histiocytosis 10.0

Graphical network of the top 20 diseases related to Bone Giant Cell Tumor:



Diseases related to Bone Giant Cell Tumor

Symptoms & Phenotypes for Bone Giant Cell Tumor

UMLS symptoms related to Bone Giant Cell Tumor:


pain

MGI Mouse Phenotypes related to Bone Giant Cell Tumor:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.86 CALCR CTSB CTSK ITGAV SPP1 THBS1
2 craniofacial MP:0005382 9.72 CTSK ITGAV SPP1 THRA TNFSF11
3 limbs/digits/tail MP:0005371 9.55 CALCR CTSK THBS1 THRA TNFSF11
4 neoplasm MP:0002006 9.43 CTSB ITGAV SPP1 THBS1 THRA TNFSF11
5 renal/urinary system MP:0005367 9.02 CTSB ITGAV SPP1 THBS1 THRA

Drugs & Therapeutics for Bone Giant Cell Tumor

Drugs for Bone Giant Cell Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 57)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Denosumab Approved Phase 4 615258-40-7
2 Antibodies Phase 4
3 Immunoglobulins Phase 4
4 Antibodies, Monoclonal Phase 4
5 Immunoglobulin G Phase 4
6
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
7
Ethanol Approved Phase 3 64-17-5 702
8
Phenol Approved, Experimental Phase 3 108-95-2 996
9 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
10 Analgesics Phase 3
11 Peripheral Nervous System Agents Phase 3
12 Cola Phase 3
13 Liver Extracts Phase 3
14 Anesthetics, Intravenous Phase 3
15 Narcotics Phase 3
16 Analgesics, Opioid Phase 3
17 Adjuvants, Anesthesia Phase 3
18 Central Nervous System Depressants Phase 3
19 Anesthetics, General Phase 3
20 Anesthetics Phase 3
21 Diphosphonates Phase 3
22 Polymethyl Methacrylate Phase 3
23
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
24
Aldesleukin Approved Phase 2 85898-30-2, 110942-02-4
25
Fludarabine Approved Phase 2 21679-14-1, 75607-67-9 30751
26
Vidarabine Approved, Investigational Phase 2 24356-66-9 21704 32326
27
Zoledronic Acid Approved Phase 2 118072-93-8 68740
28
Vitamin D Approved, Nutraceutical, Vet_approved Phase 2 1406-16-2
29
Serine Approved, Nutraceutical Phase 2 56-45-1 5951
30
Emodepside Investigational, Vet_approved Phase 2 155030-63-0
31 Micronutrients Phase 2
32 Trace Elements Phase 2
33 Hormones Phase 2
34 Vitamins Phase 2
35 Nutrients Phase 2
36 Calciferol Phase 2
37 Protein Kinase Inhibitors Phase 2
38 Analgesics, Non-Narcotic Phase 2
39 Anti-Infective Agents Phase 2
40 Vidarabine Phosphate Phase 2
41 Anti-HIV Agents Phase 2
42 Anti-Retroviral Agents Phase 2
43 Interleukin-2 Phase 2
44 Antimetabolites Phase 2
45 Antimetabolites, Antineoplastic Phase 2
46 Antiviral Agents Phase 2
47 Bone Density Conservation Agents Phase 2
48 Pharmaceutical Solutions Phase 1, Phase 2
49
Cyclophosphamide Approved, Investigational Early Phase 1 50-18-0, 6055-19-2 2907
50
Calcium Approved, Nutraceutical 7440-70-2 271

Interventional clinical trials:

(show all 16)
# Name Status NCT ID Phase Drugs
1 Long-term Safety Follow-up of Subjects With Giant Cell Tumor of Bone Treated With Denosumab in Study 20062004 Active, not recruiting NCT03301857 Phase 4 Denosumab (Cohort A)
2 Clinical, Pathologic Characteristics and Its Mechansim of Denosumab Treated Giant Cell Tumor of Bone Unknown status NCT03259152 Phase 3 Denosumab
3 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
4 Local Bisphosphonate Effect on Recurrence Rate in Extremity Giant Cell Tumor of Bone: A Prospective Randomized Controlled Trial Recruiting NCT03295981 Phase 3 Zoledronic Acid
5 An Open-Label, Multi-Center, Phase 2 Safety and Efficacy Study of Denosumab (AMG 162) in Subjects With Recurrent or Unresectable Giant Cell Tumor (GCT) of Bone Completed NCT00396279 Phase 2
6 An Open-label, Multi-center, Phase 2 Study of Denosumab in Subjects With Giant Cell Tumor of Bone Completed NCT00680992 Phase 2 Denosumab
7 A Phase II Trial of Dasatinib in Advanced Sarcomas Completed NCT00464620 Phase 2 Dasatinib
8 Denosumab: A Potential Treatment Option for Aneurysmal Bone Cysts Completed NCT03415477 Phase 1, Phase 2 Denosumab (Xgeva)
9 Clinical Study to Assess Efficacy and Safety of LN-145 (Autologous Centrally Manufactured Tumor Infiltrating Lymphocytes) Across Multiple Tumor Types Recruiting NCT03449108 Phase 2 Cyclophosphamide;Fludarabine;Fludarabine Phosphate
10 Reduced Dose-density of Denosumab for Maintenance Therapy of Unresectable Giant Cell Tumor of Bone: a Multicenter Phase II Study "REDUCE" Not yet recruiting NCT03620149 Phase 2 Denosumab 120 MG/1.7 ML Subcutaneous Solution [XGEVA]
11 Adjuvant Zoledronic Acid in 'High Risk' Giant Cell Tumour of Bone (GCT) - A Randomized Phase II Study Terminated NCT00889590 Phase 2 Zoledronic acid
12 Phase 2 Study of the Effect of Zoledronate on Local Recurrence After Surgical Treatment of Giant Cell Tumors of Bone Terminated NCT01564121 Phase 1, Phase 2 acid Zoledronic
13 Il Tumore a Cellule Giganti Delle estermità Completed NCT02996734
14 Giant Cell Tumor of the Bone in Patients Under 15 Years Old: a Single Institution Case-series Recruiting NCT04002817
15 Safety and Efficacy of Post-operative Denosumab Strategy for Giant Cell Tumor of Bone Treatment in China:Correlation Between Recurrence and Drug Withdrawl Not yet recruiting NCT03358212 Denosumab
16 Sequential Administration of Cryoablation and Cyclophosphamide for Advanced Solid Epithelial Cancer Terminated NCT00499733 Early Phase 1 cyclophosphamide

Search NIH Clinical Center for Bone Giant Cell Tumor

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Denosumab

Cochrane evidence based reviews: giant cell tumor of bone

Genetic Tests for Bone Giant Cell Tumor

Anatomical Context for Bone Giant Cell Tumor

MalaCards organs/tissues related to Bone Giant Cell Tumor:

41
Bone, Monocytes, Lung, Pancreas, Lymph Node, Breast, Liver

Publications for Bone Giant Cell Tumor

Articles related to Bone Giant Cell Tumor:

(show top 50) (show all 1049)
# Title Authors PMID Year
1
Clinical Significance of MRI and Pathological Features of Giant Cell Tumor of Bone Boundary. 38
31422584 2019
2
Absence of H3F3A mutation in a subset of malignant giant cell tumor of bone. 38
31285528 2019
3
[The study of the value of H3F3A G34W immunohistochemical staining in the diagnosis of giant cell tumor of bone]. 38
31288308 2019
4
Challenges of denosumab in giant cell tumor of bone, and other giant cell-rich tumors of bone. 38
30844887 2019
5
Adjuvant Zoledronic Acid in High-Risk Giant Cell Tumor of Bone: A Multicenter Randomized Phase II Trial. 38
31040253 2019
6
Morphologic Changes After Denosumab Therapy in Patients with Giant Cell Tumor of the Spine: Report of Four Cases and a Review of the Literature. 38
30926552 2019
7
In situ cell cycle analysis in giant cell tumor of bone reveals patients with elevated risk of reduced progression-free survival. 38
31233932 2019
8
A new computerized tomography classification to evaluate response to Denosumab in giant cell tumors in the extremities. 38
31253385 2019
9
Similar local recurrence but better function with curettage versus resection for bone giant cell tumor and pathological fracture at presentation. 38
30734307 2019
10
Intra-articular ectopic ossification associated with denosumab administration for giant cell tumor of bone with intra-articular pathological fracture. 38
28109658 2019
11
Preoperative Denosumab plus Surgery in the Management of Giant Cell Tumor of Bone: A Comprehensive Narrative Literature Review. 38
31242985 2019
12
Characterization of Three Novel H3F3A-mutated Giant Cell Tumor Cell Lines and Targeting of Their Wee1 Pathway. 38
31015476 2019
13
Knock-down of oncohistone H3F3A-G34W counteracts the neoplastic phenotype of giant cell tumor of bone derived stromal cells. 38
30742944 2019
14
Denosumab versus zoledronic acid in cases of surgically unsalvageable giant cell tumor of bone: A randomized clinical trial. 38
30740297 2019
15
Measurement of morphological parameters of giant cell tumor of bone in the knee. 38
30930988 2019
16
A randomized phase III trial of denosumab before curettage for giant cell tumor of bone: Japan Clinical Oncology Group Study JCOG1610. 38
30796832 2019
17
The prognostic significance of the neutrophil-to-lymphocyte ratio and the platelet-to-lymphocyte ratio in giant cell tumor of the extremities. 38
30961549 2019
18
Treatment of Massive Giant Cell Tumor of Bone of the Proximal Radius. Case Study. 38
31180037 2019
19
Giant Cell Tumor of Bone: Review of Current Literature, Evaluation, and Treatment Options. 38
30449024 2019
20
An unusual case of giant cell tumor of soft parts in an American Warmblood horse. 38
31086765 2019
21
Giant Cell Tumor of the Carotid Body: A Rare Tumor in a Novel Location. 38
30922105 2019
22
Bisphosphonate-loaded Bone Cement as a Local Adjuvant Therapy for Giant Cell Tumor of Bone: A 1 to 12-Year Follow-up Study. 38
30811352 2019
23
Convolutional neural network to predict the local recurrence of giant cell tumor of bone after curettage based on pre-surgery magnetic resonance images. 38
30859281 2019
24
Denosumab treatment for giant-cell tumor of bone: a systematic review of the literature. 38
30877429 2019
25
Tumor immunohistochemistry and preoperative magnetic resonance imaging features predict local recurrence of giant cell tumor of bone following intralesional curettage. 38
30675196 2019
26
Managements of giant cell tumor within the distal radius: A retrospective study of 58 cases from a single center. 38
30705814 2019
27
Recurrent Adolescent Giant-Cell Tumor of the Scaphoid: Scaphoid Excision with Intracarpal Fusion after Failed Curettage and Bone Grafting. 38
31093399 2019
28
Effect of bisphosphonates on local recurrence of giant cell tumor of bone: a meta-analysis. 38
30666162 2019
29
In Vitro Study of the Effects of Denosumab on Giant Cell Tumor of Bone: Comparison with Zoledronic Acid. 38
29159783 2019
30
Histopathology of giant cell tumors of the bone: With special emphasis on fibrohistiocytic and aneurysmal bone cyst like components. 38
30392920 2019
31
Malignancy in Giant Cell Tumor of Bone: A Review of the Literature. 38
30935298 2019
32
Malignant Sarcomatous Transformation of Benign Giant Cell Tumor of Bone after Treatment with Denosumab Therapy: A Literature Review of Reported Cases. 38
30868006 2018
33
Role of Bisphosphonates as Adjuvants of Surgery in Giant Cell Tumor of Spine. 38
30619673 2018
34
Secondary malignant giant cell tumor of bone with histone H3.3 mutation: A case series. 38
30522923 2018
35
Neoadjuvant and Adjuvant Treatment with Denosumab in Aggressive Giant-cell Tumor of Bone in the Proximal Fibula: a Case Report. 38
31188771 2018
36
MHRA Drug Safety Update: denosumab (Xgeva) and rebound hypercalcaemia. 38
30297446 2018
37
A novel subchondral bone-grafting procedure for the treatment of giant-cell tumor around the knee: A retrospective study of 27 cases. 38
30407342 2018
38
Long-term Follow-up of the Use of a Synthetic Bone Graft Composite in the Surgical Management of Primary Bone Tumors. 38
30371921 2018
39
Giant cell tumor of bone: updated molecular pathogenesis and tumor biology. 38
29944971 2018
40
Diagnostic Immunohistochemistry for Soft Tissue and Bone Tumors: An Update. 38
30134255 2018
41
Intratibial injection of patient-derived tumor cells from giant cell tumor of bone elicits osteolytic reaction in nude mouse. 38
30214599 2018
42
Denosumab in patients with aneurysmal bone cysts: A case series with preliminary results. 38
30086700 2018
43
Primary thoracic giant cell tumor of bone sensitive to steroids. 38
29502130 2018
44
Incidence and demographics of giant cell tumor of bone in The Netherlands: First nationwide Pathology Registry Study. 38
29987945 2018
45
Giant Cell Tumor of the Frontal Bone: A Case Report and Literature Review. 38
30483457 2018
46
Assessment of denosumab treatment effects and imaging response in patients with giant cell tumor of bone. 38
30231890 2018
47
Epidemiology of benign giant cell tumor of bone in the Chinese population. 38
30148063 2018
48
Management of an Unusual Periprosthetic Giant Cell Tumor of Bone of the Proximal Tibia. 38
30465034 2018
49
An extremely rare case of primary malignancy in giant cell tumor of bone, arising in the right femur and harboring H3F3A mutation. 38
29970305 2018
50
[H3F3A mutated multicentric giant cell tumor of bone : A very rare primary bone disease]. 38
30046846 2018

Variations for Bone Giant Cell Tumor

Expression for Bone Giant Cell Tumor

Search GEO for disease gene expression data for Bone Giant Cell Tumor.

Pathways for Bone Giant Cell Tumor

GO Terms for Bone Giant Cell Tumor

Cellular components related to Bone Giant Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.7 TNFSF11 THBS1 SPP1 SERPINA3 CTSK CTSB
2 extracellular space GO:0005615 9.5 TNFSF11 THBS1 SPP1 SERPINA3 CTSK CTSB
3 endolysosome lumen GO:0036021 8.62 CTSK CTSB

Biological processes related to Bone Giant Cell Tumor according to GeneCards Suite gene sharing:

(show all 20)
# Name GO ID Score Top Affiliating Genes
1 extracellular matrix organization GO:0030198 9.72 THBS1 SPP1 ITGAV
2 positive regulation of protein kinase B signaling GO:0051897 9.65 TNFSF11 THBS1 CALCR
3 positive regulation of cytosolic calcium ion concentration GO:0007204 9.61 ITGAV CALCR CALCA
4 monocyte chemotaxis GO:0002548 9.59 TNFSF11 CALCA
5 embryo implantation GO:0007566 9.58 SPP1 CALCA
6 negative regulation of extrinsic apoptotic signaling pathway GO:2001237 9.57 THBS1 ITGAV
7 collagen catabolic process GO:0030574 9.56 CTSK CTSB
8 activation of adenylate cyclase activity GO:0007190 9.54 CALCR CALCA
9 osteoclast differentiation GO:0030316 9.52 TNFSF11 CALCR
10 positive regulation of phosphorylation GO:0042327 9.51 TNFSF11 THBS1
11 inflammatory response GO:0006954 9.5 THBS1 SPP1 SERPINA3
12 ERK1 and ERK2 cascade GO:0070371 9.49 TNFSF11 ITGAV
13 decidualization GO:0046697 9.46 SPP1 CTSB
14 positive regulation of bone resorption GO:0045780 9.37 TNFSF11 SPP1
15 ossification GO:0001503 9.33 TNFSF11 THRA ACP5
16 regulation of heart contraction GO:0008016 9.27 THRA
17 negative regulation of interleukin-12 production GO:0032695 9.26 THBS1 ACP5
18 negative regulation of ossification GO:0030279 9.1 CALCR
19 amylin receptor signaling pathway GO:0097647 8.96 CALCR CALCA
20 bone resorption GO:0045453 8.8 TNFSF11 CTSK ACP5

Molecular functions related to Bone Giant Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular matrix binding GO:0050840 9.33 THBS1 SPP1 ITGAV
2 fibroblast growth factor binding GO:0017134 9.32 THBS1 ITGAV
3 transforming growth factor beta binding GO:0050431 9.26 THBS1 ITGAV
4 fibronectin binding GO:0001968 9.13 THBS1 ITGAV CTSK
5 proteoglycan binding GO:0043394 8.8 THBS1 CTSK CTSB
6 protein binding GO:0005515 10.11 TNFSF11 THRA THBS1 SPP1 SERPINA3 ITGAV

Sources for Bone Giant Cell Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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