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MCID: TNS001
MIFTS: 39

Tenosynovial Giant Cell Tumor

Categories: Cancer diseases, Muscle diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Tenosynovial Giant Cell Tumor

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MalaCards integrated aliases for Tenosynovial Giant Cell Tumor:

Name: Tenosynovial Giant Cell Tumor 12 15 17
Fibrous Histiocytoma of Tendon Sheath 12 74
Giant Cell Tumor of Tendon Sheath 12
Giant Cell Tumor of Tenosynovium 12

Classifications:

MalaCards categories:
Global: Cancer diseases
Anatomical: Muscle diseases
See all MalaCards categories (disease lists)


External Ids:

Disease Ontology 12 DOID:314
ICD9CM 36 727.02
MeSH 45 D000070779
NCIt 51 C3402
UMLS 74 C1318543
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Summaries for Tenosynovial Giant Cell Tumor

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MalaCards based summary : Tenosynovial Giant Cell Tumor, also known as fibrous histiocytoma of tendon sheath, is related to pigmented villonodular synovitis and giant cell tumor. An important gene associated with Tenosynovial Giant Cell Tumor is CSF1 (Colony Stimulating Factor 1), and among its related pathways/superpathways are Pathways in cancer and Hematopoietic cell lineage. The drugs Sargramostim and Grapefruit Seed Extract have been mentioned in the context of this disorder. Affiliated tissues include bone, brain and thyroid, and related phenotype is no phenotypic analysis.

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Related Diseases for Tenosynovial Giant Cell Tumor

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

(show top 50) (show all 51)
# Related Disease Score Top Affiliating Genes
1 pigmented villonodular synovitis 33.1 CSF1 CSF1R
2 giant cell tumor 31.8 CSF1 SERPINA3
3 synovitis 10.6
4 villonodular synovitis 10.6
5 patulous eustachian tube 10.3
6 hemangioma 10.2
7 bone giant cell tumor 10.2
8 sclerosing hemangioma 10.2
9 noonan syndrome 1 10.2
10 nodular tenosynovitis 10.2
11 tenosynovitis 10.2
12 pseudo-turner syndrome 10.2
13 histiocytoma 10.2
14 fibrous histiocytoma 10.2
15 histiocytic sarcoma 10.2 CDKN2A CSF1
16 atypical neurofibroma 10.1 CDKN2A CDKN2B
17 ewing's family of tumors 10.1 CDKN2A CDKN2B
18 acute lymphoblastic leukemia, childhood 10.1 CDKN2A CDKN2B
19 carpal tunnel syndrome 10.1
20 lipomatosis, multiple 10.1
21 mononeuropathy of the median nerve, mild 10.1
22 pleomorphic lipoma 10.1
23 malignant giant cell tumor 10.1
24 enchondroma 10.1
25 pyogenic granuloma 10.1
26 grade iii astrocytoma 10.1 CDKN2A CDKN2B
27 skin melanoma 10.1 CDKN2A CDKN2B
28 fibrosarcoma of bone 10.1 CDKN2A SERPINA3
29 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 10.1 CSF1 CSF1R
30 neurofibromatosis, type i 10.1
31 neurofibromatosis, type iv, of riccardi 10.1
32 rheumatoid arthritis 10.1
33 sarcoma, synovial 10.1
34 arthritis 10.1
35 fibroma 10.1
36 lymphoma 10.1
37 sarcoma 10.1
38 thyroid cancer 10.1
39 spindle cell sarcoma 10.1
40 leukemia, acute lymphoblastic 3 10.0 CDKN2A CDKN2B
41 nervous system cancer 10.0 CDKN2A CDKN2B
42 histiocytosis 10.0 CSF1R SERPINA3
43 acute lymphocytic leukemia 9.9 CDKN2A CDKN2B
44 precursor t-cell acute lymphoblastic leukemia 9.9 CDKN2A CDKN2B
45 scrotal carcinoma 9.8 CDKN2A CDKN2B SERPINA3
46 retinal hemangioblastoma 9.8 CLU SERPINA3
47 hematologic cancer 9.8 CDKN2B CSF1 CSF1R
48 malignant histiocytosis 9.8 CSF1 CSF1R SERPINA3
49 myelodysplastic syndrome 9.7 CDKN2B CSF1 CSF1R
50 anaplastic large cell lymphoma 9.7 CLU CSF1R

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



Diseases related to Tenosynovial Giant Cell Tumor
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Symptoms & Phenotypes for Tenosynovial Giant Cell Tumor

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MGI Mouse Phenotypes related to Tenosynovial Giant Cell Tumor:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 no phenotypic analysis MP:0003012 8.92 CDKN2A CDKN2B CSF1 CSF1R
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Drugs & Therapeutics for Tenosynovial Giant Cell Tumor

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

(show all 23)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sargramostim Approved, Investigational Phase 3 123774-72-1, 83869-56-1
2 Grapefruit Seed Extract Phase 2
3 Immunoglobulins Phase 1, Phase 2
4 Antibodies Phase 1, Phase 2
5
Paclitaxel Approved, Vet_approved Phase 1 33069-62-4 36314
6
Midazolam Approved, Illicit Phase 1 59467-70-8 4192
7
Tolbutamide Approved, Investigational Phase 1 64-77-7 5505
8 Albumin-Bound Paclitaxel Phase 1
9 Antimitotic Agents Phase 1
10 Antineoplastic Agents, Phytogenic Phase 1
11 Central Nervous System Depressants Phase 1
12 Hypoglycemic Agents Phase 1
13 Psychotropic Drugs Phase 1
14 Neurotransmitter Agents Phase 1
15 Anesthetics Phase 1
16 Anesthetics, Intravenous Phase 1
17 Anesthetics, General Phase 1
18 GABA Agents Phase 1
19 Hypnotics and Sedatives Phase 1
20 Tranquilizing Agents Phase 1
21 Anti-Anxiety Agents Phase 1
22 Adjuvants, Anesthesia Phase 1
23 GABA Modulators Phase 1

Interventional clinical trials:

(show all 11)
# Name Status NCT ID Phase Drugs
1 Phase 3 Study of Pexidartinib for Pigmented Villonodular Synovitis (PVNS) or Giant Cell Tumor of the Tendon Sheath (GCT-TS) Active, not recruiting NCT02371369 Phase 3 Pexidartinib;Placebo
2 Study of Nilotinib Efficacy in Pigmented Villo-Nodular Synovitis/ Tenosynovial Giant Cell Tumour (PVNS/TGCT) Completed NCT01261429 Phase 2 Tasigna
3 MCS110 in Patients With Pigmented Villonodular Synovitis (PVNS) Completed NCT01643850 Phase 2 MCS110;Placebo;MCS110;Placebo
4 Study of Cabiralizumab in Patients With Pigmented Villonodular Synovitis / Diffuse Type Tenosynovial Giant Cell Tumor Active, not recruiting NCT02471716 Phase 1, Phase 2
5 Nilotinib in Patients With Relapsed or Metastatic Pigmented Villonodular Synovitis/Tenosynovial Giant Cell Tumor/Diffuse-Type Giant Cell Tumor Active, not recruiting NCT01207492 Phase 2 nilotinib
6 A Study of RO5509554 as Monotherapy and in Combination With Paclitaxel in Participants With Advanced Solid Tumors Completed NCT01494688 Phase 1 Paclitaxel;RO5509554
7 Effect of Pexidartinib on the Way the Body Processes CYP3A4 and CYP2C9 Substrates (Pharmacokinetics) Active, not recruiting NCT03291288 Phase 1 Tolbutamide;Midazolam;Pexidartinib
8 A Study of PLX73086 in Advanced Solid Tumors and Locally Advanced or Refractory Tenosynovial Giant Cell Tumor Terminated NCT02673736 Phase 1 PLX73086
9 Phase 1 Study of PLX7486 as Single Agent in Patients With Advanced Solid Tumors Terminated NCT01804530 Phase 1 PLX7486 TsOH
10 Tenosynovial Giant Cell Tumors (TGCT) Observational Platform Project Recruiting NCT02948088
11 Evaluation of the Effectiveness of Surgical Treatment of Villo-nodular Synovitis of the Hip in Children Recruiting NCT03507933

Search NIH Clinical Center for Tenosynovial Giant Cell Tumor

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Genetic Tests for Tenosynovial Giant Cell Tumor

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Anatomical Context for Tenosynovial Giant Cell Tumor

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MalaCards organs/tissues related to Tenosynovial Giant Cell Tumor:

42
Bone, Brain, Thyroid, Salivary Gland
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Publications for Tenosynovial Giant Cell Tumor

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Articles related to Tenosynovial Giant Cell Tumor:

(show top 50) (show all 94)
# Title Authors Year
1
Diffuse-Type Tenosynovial Giant Cell Tumor of the Shoulder Evaluated by FDG PET/CT. ( 30624274 )
2019
2
The measurement of physical functioning among patients with Tenosynovial Giant Cell Tumor (TGCT) using the Patient-Reported Outcomes Measurement Information System (PROMIS). ( 30714073 )
2019
3
Diffuse Tenosynovial Giant Cell Tumor Around the Temporomandibular Joint: An Entity With Special Radiologic and Pathologic Features. ( 30796908 )
2019
4
Diffuse-Type Tenosynovial Giant Cell Tumor of the Thoracic Spine: Appearance on FDG PET/CT. ( 30889006 )
2019
5
Distinct extra-articular invasion patterns of diffuse pigmented villonodular synovitis/tenosynovial giant cell tumor in the knee joints. ( 29637236 )
2018
6
Extra-articular tenosynovial giant cell tumor of diffuse type in the temporal area with brain parenchymal invasion: a case report. ( 29334779 )
2018
7
Successful treatment of a diffuse type tenosynovial giant cell tumor in the thoracic spine mimicking spinal metastasis by frozen recapping laminoplasty in a patient with thyroid cancer. ( 29663145 )
2018
8
Intra-articular fibroma-like perivascular epithelioid tumor (PEComa) mimicking tenosynovial giant cell tumor, diffuse type. ( 30206676 )
2018
9
Hot shoulder PET/CT lesion: Unusual presentation of tenosynovial giant cell tumor. ( 29988775 )
2018
10
Tenosynovial giant cell tumor: case report of a patient effectively treated with pexidartinib (PLX3397) and review of the literature. ( 30002809 )
2018
11
Giant Cell Poor Extra-Articular Diffuse-Type Tenosynovial Giant Cell Tumor With Extensive Desmin Expression: A Potential Diagnostic Pitfall With Cytogenetic Confirmation. ( 30019981 )
2018
12
Tenosynovial Giant Cell Tumor of the Clinoid: Rare Condition. ( 30026165 )
2018
13
Malignant Tenosynovial Giant Cell Tumor: The True "Synovial Sarcoma?" A Clinicopathologic, Immunohistochemical, and Molecular Cytogenetic Study of 10 Cases, Supporting Origin from Synoviocytes. ( 30206409 )
2018
14
Desmoplastic fibroblastoma mimicking tenosynovial giant cell tumor encasing a tendon of the foot. ( 30229348 )
2018
15
Bone Involvement Mimicking an Aggressive Bone Lesion in a Diffuse-type Tenosynovial Giant Cell Tumor in the Thoracic Vertebral Lamina: A Case Report. ( 30584508 )
2018
16
Clinical characteristics and treatment outcomes in six cases of malignant tenosynovial giant cell tumor: initial experience of molecularly targeted therapy. ( 30594158 )
2018
17
Concomitant Spondyloarthritis and Tenosynovial Giant Cell Tumor in Pigmented Villonodular Synovitis Challenging Cases. ( 30601200 )
2018
18
Chondroid Tenosynovial Giant Cell Tumor of Temporomandibular Joint. ( 30693256 )
2018
19
Extra-articular diffuse-type tenosynovial giant cell tumor with benign histological features resulting in fatal pulmonary metastases. ( 28219303 )
2017
20
Chondroid Tenosynovial Giant Cell Tumor of the Temporomandibular Joint: A Rare Case Report. ( 29215692 )
2017
21
Imaging features for diffuse-type tenosynovial giant cell tumor of the temporomandibular joint: A case report. ( 28658169 )
2017
22
Localized and diffuse forms of tenosynovial giant cell tumor (formerly giant cell tumor of the tendon sheath and pigmented villonodular synovitis). ( 28057477 )
2017
23
Tenosynovial Giant Cell Tumor: Incidence, Prevalence, Patient Characteristics, and Recurrence. A Registry-based Cohort Study in Denmark. ( 28765257 )
2017
24
Tenosynovial Giant Cell Tumor in the Midfoot Treated With Femoral Head Allograft Reconstruction. ( 28864387 )
2017
25
Pitfalls in the cytological diagnosis of tenosynovial giant cell tumor: An illustration of eight discordant cases. ( 28941190 )
2017
26
Prognosis of Advanced Tenosynovial Giant Cell Tumor of the Knee Diagnosed During Total Knee Arthroplasty. ( 28161138 )
2017
27
Fine needle aspiration cytology diagnosis of metastatic malignant diffuse type tenosynovial giant cell tumor. ( 28701836 )
2017
28
Tenosynovial Giant Cell Tumor in the Dermis of the External Auditory Meatus. ( 27072732 )
2016
29
Coexisting sarcoidal granulomatous inflammation and diffuse tenosynovial giant cell tumor of the knee after a total knee replacement: a case report. ( 27717976 )
2016
30
Pediatric Intra-Articular Localized Tenosynovial Giant Cell Tumor Presenting as an Acutely Irritable Hip: A Case Report. ( 29252637 )
2016
31
Current Systemic Treatment Options for Tenosynovial Giant Cell Tumor/Pigmented Villonodular Synovitis: Targeting the CSF1/CSF1R Axis. ( 26820289 )
2016
32
Intramuscular Tenosynovial Giant Cell Tumor, Diffuse-Type. ( 26755356 )
2016
33
Diffuse Type Tenosynovial Giant Cell Tumor of the Ankle. ( 26996490 )
2016
34
Malignant tenosynovial giant cell tumor with CDKN2A/B genomic alteration: a histological, immunohistochemical, and molecular study. ( 27989786 )
2016
35
Tenosynovial giant cell tumor arising from the posterior cruciate ligament: a case report and literature review. ( 26261570 )
2015
36
Fine Needle Aspiration Cytology of Chondroid Tenosynovial Giant Cell Tumor of the Hand. ( 26266013 )
2015
37
Structure-Guided Blockade of CSF1R Kinase in Tenosynovial Giant-Cell Tumor. ( 26222558 )
2015
38
Tenosynovial Giant Cell Tumor, Diffuse Type/Pigmented Villonodular Synovitis in a Pars Defect: A Case Report. ( 26067150 )
2015
39
Arthroscopic Resection of a Tenosynovial Giant Cell Tumor in the Wrist: A Case Report. ( 26496348 )
2015
40
Malignant tenosynovial giant cell tumor of the leg: a radiologic-pathologic correlation and review of the literature. ( 25861547 )
2015
41
Intra-articular tenosynovial giant cell tumor arising from the posterior cruciate ligament. ( 26337311 )
2015
42
Tenosynovial Giant Cell Tumor of Diffuse Type Mimicking Bony Metastasis Detected on F-18 FDG PET/CT. ( 25177381 )
2014
43
Primary diffuse-type tenosynovial giant cell tumor of the spine: a report of 3 cases and systemic review of the literature. ( 25269059 )
2014
44
A tenosynovial giant cell tumor arising from femoral attachment of the anterior cruciate ligament. ( 24900909 )
2014
45
Hepatoid tenosynovial giant cell tumor - A rare morphologic variant case report. ( 25023880 )
2014
46
Diffuse-type tenosynovial giant cell tumor with t(1;17)(p13;p13) and trisomy 5. ( 25189912 )
2014
47
Intramuscular diffuse-type tenosynovial giant cell tumor of the deltoid muscle in a child. ( 24676801 )
2014
48
Tenosynovial giant cell tumor presenting as a parotid gland mass: Expanding the differential diagnosis of giant cell-rich lesions in salivary glands. ( 25745294 )
2014
49
Multiple metastases from histologically benign intraarticular diffuse-type tenosynovial giant cell tumor: a case report. ( 25130396 )
2014
50
Recurrent temporal bone tenosynovial giant cell tumor with chondroid metaplasia: the use of imaging to assess recurrence. ( 24571839 )
2014
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Variations for Tenosynovial Giant Cell Tumor

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Expression for Tenosynovial Giant Cell Tumor

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Search GEO for disease gene expression data for Tenosynovial Giant Cell Tumor.
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Pathways for Tenosynovial Giant Cell Tumor

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Pathways related to Tenosynovial Giant Cell Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Pathways in cancer 38
12.36 CDKN2A CDKN2B CSF1R
2
Hematopoietic cell lineage 38
11.46 CSF1 CSF1R
3
Dendritic Cells Developmental Lineage Pathway 66
11.3 CSF1 CSF1R
4
Validated targets of C-MYC transcriptional repression 1
11.19 CDKN2B CLU
5
Macrophage Differentiation and Growth Inhibition by METS 65
Show member pathways
 11.08 CSF1 CSF1R
6
Signaling events mediated by PTP1B 1
11.02 CSF1 CSF1R
7
Differentiation Pathway 1
10.94 CSF1 CSF1R
8
Integrins in angiogenesis 1
10.82 CSF1 CSF1R
9
Signaling events mediated by TCPTP 1
Show member pathways
 10.58 CSF1 CSF1R
10
Oncogene Induced Senescence 67
10.13 CDKN2A CDKN2B
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GO Terms for Tenosynovial Giant Cell Tumor

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

# Name GO ID Score Top Affiliating Genes
1 platelet alpha granule lumen GO:0031093 8.96 CLU SERPINA3
2 CSF1-CSF1R complex GO:1990682 8.62 CSF1 CSF1R

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

# Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.63 CLU CSF1 CSF1R
2 negative regulation of cell proliferation GO:0008285 9.58 CDKN2A CDKN2B CSF1R
3 positive regulation of gene expression GO:0010628 9.54 CDKN2A CLU CSF1
4 inflammatory response GO:0006954 9.5 CSF1 CSF1R SERPINA3
5 hemopoiesis GO:0030097 9.43 CSF1 CSF1R
6 osteoclast differentiation GO:0030316 9.26 CSF1 CSF1R
7 macrophage differentiation GO:0030225 9.16 CSF1 CSF1R
8 negative regulation of phosphorylation GO:0042326 8.96 CDKN2A CDKN2B
9 macrophage colony-stimulating factor signaling pathway GO:0038145 8.62 CSF1 CSF1R

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

# Name GO ID Score Top Affiliating Genes
1 cyclin-dependent protein serine/threonine kinase inhibitor activity GO:0004861 8.62 CDKN2A CDKN2B
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Sources for Tenosynovial Giant Cell Tumor

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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