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MCID: TNS001
MIFTS: 37

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
Fibrous Histiocytoma of Tendon Sheath 12 73
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 35 727.02
MeSH 44 D000070779
NCIt 50 C3402
UMLS 73 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 Dendritic Cells Developmental Lineage Pathway and Validated targets of C-MYC transcriptional repression. The drugs Grapefruit Seed Extract and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include bone, brain and thyroid.

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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 all 43)
# Related Disease Score Top Affiliating Genes
1 pigmented villonodular synovitis 33.2 CSF1 CSF1R
2 giant cell tumor 31.7 CSF1 SERPINA3
3 synovitis 10.6
4 villonodular synovitis 10.6
5 hemangioma 10.2
6 bone giant cell tumor 10.2
7 sclerosing hemangioma 10.2
8 noonan syndrome 1 10.2
9 nodular tenosynovitis 10.2
10 tenosynovitis 10.2
11 pseudo-turner syndrome 10.2
12 histiocytoma 10.2
13 fibrous histiocytoma 10.2
14 atypical neurofibroma 10.1 CDKN2A CDKN2B
15 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 10.1 CSF1 CSF1R
16 ewing's family of tumors 10.1 CDKN2A CDKN2B
17 acute lymphoblastic leukemia, childhood 10.1 CDKN2A CDKN2B
18 carpal tunnel syndrome 10.1
19 mononeuropathy of the median nerve, mild 10.1
20 malignant giant cell tumor 10.1
21 pyogenic granuloma 10.1
22 grade iii astrocytoma 10.1 CDKN2A CDKN2B
23 skin melanoma 10.1 CDKN2A CDKN2B
24 leukemia, acute lymphoblastic 3 10.1 CDKN2A CDKN2B
25 fibrosarcoma of bone 10.0 CDKN2A SERPINA3
26 histiocytosis 10.0 CSF1R SERPINA3
27 neurofibromatosis, type i 10.0
28 rheumatoid arthritis 10.0
29 sarcoma, synovial 10.0
30 arthritis 10.0
31 lymphoma 10.0
32 sarcoma 10.0
33 thyroid cancer 10.0
34 spindle cell sarcoma 10.0
35 retinal hemangioblastoma 10.0 CLU SERPINA3
36 nervous system cancer 10.0 CDKN2A CDKN2B
37 hematologic cancer 9.9 CDKN2B CSF1 CSF1R
38 scrotal carcinoma 9.9 CDKN2A CDKN2B SERPINA3
39 anaplastic large cell lymphoma 9.9 CLU CSF1R
40 malignant histiocytosis 9.9 CSF1 CSF1R SERPINA3
41 acute lymphocytic leukemia 9.9 CDKN2A CDKN2B
42 myelodysplastic syndrome 9.9 CDKN2B CSF1 CSF1R
43 dermatofibrosarcoma protuberans 9.8 COL6A3 SERPINA3

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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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 22)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Grapefruit Seed Extract Phase 2
2 Immunoglobulins Phase 1, Phase 2
3 Antibodies Phase 1, Phase 2
4
Paclitaxel Approved, Vet_approved Phase 1 33069-62-4 36314
5
Tolbutamide Approved, Investigational Phase 1 64-77-7 5505
6
Midazolam Approved, Illicit Phase 1 59467-70-8 4192
7 Antineoplastic Agents, Phytogenic Phase 1
8 Albumin-Bound Paclitaxel Phase 1
9 Antimitotic Agents Phase 1
10 Hypnotics and Sedatives Phase 1
11 Anti-Anxiety Agents Phase 1
12 Hypoglycemic Agents Phase 1
13 Central Nervous System Depressants Phase 1
14 GABA Agents Phase 1
15 Neurotransmitter Agents Phase 1
16 Anesthetics, General Phase 1
17 Anesthetics, Intravenous Phase 1
18 Tranquilizing Agents Phase 1
19 Anesthetics Phase 1
20 Adjuvants, Anesthesia Phase 1
21 Psychotropic Drugs Phase 1
22 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 Study of Cabiralizumab in Patients With Pigmented Villonodular Synovitis / Diffuse Type Tenosynovial Giant Cell Tumor Active, not recruiting NCT02471716 Phase 1, Phase 2
4 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
5 MCS110 in Patients With Pigmented Villonodular Synovitis (PVNS) Active, not recruiting NCT01643850 Phase 2 MCS110;Placebo;MCS110;Placebo
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:

41
Bone, Brain, Thyroid, Skin, T Cells, Myeloid, Bone Marrow
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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 86)
# Title Authors Year
1
Distinct extra-articular invasion patterns of diffuse pigmented villonodular synovitis/tenosynovial giant cell tumor in the knee joints. ( 29637236 )
2018
2
Extra-articular tenosynovial giant cell tumor of diffuse type in the temporal area with brain parenchymal invasion: a case report. ( 29334779 )
2018
3
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
4
Hot shoulder PET/CT lesion: Unusual presentation of tenosynovial giant cell tumor. ( 29988775 )
2018
5
Tenosynovial giant cell tumor: case report of a patient effectively treated with pexidartinib (PLX3397) and review of the literature. ( 30002809 )
2018
6
Giant Cell Poor Extra-Articular Diffuse-Type Tenosynovial Giant Cell Tumor With Extensive Desmin Expression: A Potential Diagnostic Pitfall With Cytogenetic Confirmation. ( 30019981 )
2018
7
Tenosynovial Giant Cell Tumor of the Clinoid: Rare Condition. ( 30026165 )
2018
8
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
9
Intra-articular fibroma-like perivascular epithelioid tumor (PEComa) mimicking tenosynovial giant cell tumor, diffuse type. ( 30206676 )
2018
10
Desmoplastic fibroblastoma mimicking tenosynovial giant cell tumor encasing a tendon of the foot. ( 30229348 )
2018
11
Extra-articular diffuse-type tenosynovial giant cell tumor with benign histological features resulting in fatal pulmonary metastases. ( 28219303 )
2017
12
Chondroid Tenosynovial Giant Cell Tumor of the Temporomandibular Joint: A Rare Case Report. ( 29215692 )
2017
13
Imaging features for diffuse-type tenosynovial giant cell tumor of the temporomandibular joint: A case report. ( 28658169 )
2017
14
Localized and diffuse forms of tenosynovial giant cell tumor (formerly giant cell tumor of the tendon sheath and pigmented villonodular synovitis). ( 28057477 )
2017
15
Tenosynovial Giant Cell Tumor: Incidence, Prevalence, Patient Characteristics, and Recurrence. A Registry-based Cohort Study in Denmark. ( 28765257 )
2017
16
Tenosynovial Giant Cell Tumor in the Midfoot Treated With Femoral Head Allograft Reconstruction. ( 28864387 )
2017
17
Pitfalls in the cytological diagnosis of tenosynovial giant cell tumor: An illustration of eight discordant cases. ( 28941190 )
2017
18
Prognosis of Advanced Tenosynovial Giant Cell Tumor of the Knee Diagnosed During Total Knee Arthroplasty. ( 28161138 )
2017
19
Malignant tenosynovial giant cell tumor with CDKN2A/B genomic alteration: a histological, immunohistochemical, and molecular study. ( 27989786 )
2017
20
Fine needle aspiration cytology diagnosis of metastatic malignant diffuse type tenosynovial giant cell tumor. ( 28701836 )
2017
21
Tenosynovial Giant Cell Tumor in the Dermis of the External Auditory Meatus. ( 27072732 )
2016
22
Coexisting sarcoidal granulomatous inflammation and diffuse tenosynovial giant cell tumor of the knee after a total knee replacement: a case report. ( 27717976 )
2016
23
Pediatric Intra-Articular Localized Tenosynovial Giant Cell Tumor Presenting as an Acutely Irritable Hip: A Case Report. ( 29252637 )
2016
24
Current Systemic Treatment Options for Tenosynovial Giant Cell Tumor/Pigmented Villonodular Synovitis: Targeting the CSF1/CSF1R Axis. ( 26820289 )
2016
25
Intramuscular Tenosynovial Giant Cell Tumor, Diffuse-Type. ( 26755356 )
2016
26
Diffuse Type Tenosynovial Giant Cell Tumor of the Ankle. ( 26996490 )
2016
27
Tenosynovial giant cell tumor arising from the posterior cruciate ligament: a case report and literature review. ( 26261570 )
2015
28
Fine Needle Aspiration Cytology of Chondroid Tenosynovial Giant Cell Tumor of the Hand. ( 26266013 )
2015
29
Structure-Guided Blockade of CSF1R Kinase in Tenosynovial Giant-Cell Tumor. ( 26222558 )
2015
30
Tenosynovial Giant Cell Tumor, Diffuse Type/Pigmented Villonodular Synovitis in a Pars Defect: A Case Report. ( 26067150 )
2015
31
Arthroscopic Resection of a Tenosynovial Giant Cell Tumor in the Wrist: A Case Report. ( 26496348 )
2015
32
Malignant tenosynovial giant cell tumor of the leg: a radiologic-pathologic correlation and review of the literature. ( 25861547 )
2015
33
Intra-articular tenosynovial giant cell tumor arising from the posterior cruciate ligament. ( 26337311 )
2015
34
Tenosynovial Giant Cell Tumor of Diffuse Type Mimicking Bony Metastasis Detected on F-18 FDG PET/CT. ( 25177381 )
2014
35
Primary diffuse-type tenosynovial giant cell tumor of the spine: a report of 3 cases and systemic review of the literature. ( 25269059 )
2014
36
A tenosynovial giant cell tumor arising from femoral attachment of the anterior cruciate ligament. ( 24900909 )
2014
37
Hepatoid tenosynovial giant cell tumor - A rare morphologic variant case report. ( 25023880 )
2014
38
Diffuse-type tenosynovial giant cell tumor with t(1;17)(p13;p13) and trisomy 5. ( 25189912 )
2014
39
Intramuscular diffuse-type tenosynovial giant cell tumor of the deltoid muscle in a child. ( 24676801 )
2014
40
Tenosynovial giant cell tumor presenting as a parotid gland mass: Expanding the differential diagnosis of giant cell-rich lesions in salivary glands. ( 25745294 )
2014
41
Multiple metastases from histologically benign intraarticular diffuse-type tenosynovial giant cell tumor: a case report. ( 25130396 )
2014
42
Recurrent temporal bone tenosynovial giant cell tumor with chondroid metaplasia: the use of imaging to assess recurrence. ( 24571839 )
2014
43
Partial response of a rare malignant metastatic diffuse tenosynovial giant cell tumor with benign histologic features, treated with SCH 717-454, an insulin growth factor receptor inhibitor, in combination with everolimus, an MTOR inhibitor. ( 23430345 )
2013
44
Tenosynovial giant cell tumor arising on the scapular region. ( 24403889 )
2013
45
Tenosynovial giant cell tumor in an unusual localization. ( 23619549 )
2013
46
Chondroid tenosynovial giant cell tumor of the temporal bone. ( 23303171 )
2013
47
Navigation-guided resection for a tenosynovial giant cell tumor involving the temporomandibular joint and skull base. ( 22421861 )
2012
48
Intra-articular tenosynovial giant cell tumor arising from the posterior cruciate ligament. ( 22784912 )
2012
49
Efficacy of imatinib mesylate for the treatment of locally advanced and/or metastatic tenosynovial giant cell tumor/pigmented villonodular synovitis. ( 21823110 )
2012
50
Long-term outcome of the treatment of high-risk tenosynovial giant cell tumor/pigmented villonodular synovitis with radiotherapy and surgery. ( 22281719 )
2012
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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
Dendritic Cells Developmental Lineage Pathway 65
11.3 CSF1 CSF1R
2
Validated targets of C-MYC transcriptional repression 1
11.19 CDKN2B CLU
3
Macrophage Differentiation and Growth Inhibition by METS 64
Show member pathways
 11.08 CSF1 CSF1R
4
Signaling events mediated by PTP1B 1
11.02 CSF1 CSF1R
5
Differentiation Pathway 1
10.94 CSF1 CSF1R
6
Integrins in angiogenesis 1
10.82 CSF1 CSF1R
7
Signaling events mediated by TCPTP 1
Show member pathways
 10.58 CSF1 CSF1R
8
Oncogene Induced Senescence 66
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 extracellular region GO:0005576 9.65 CLU COL6A3 CSF1 PGM1 SERPINA3
2 platelet alpha granule lumen GO:0031093 9.16 CLU SERPINA3
3 collagen-containing extracellular matrix GO:0062023 9.13 CLU COL6A3 SERPINA3
4 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 negative regulation of cell proliferation GO:0008285 9.63 CDKN2A CDKN2B CSF1R
2 positive regulation of gene expression GO:0010628 9.61 CDKN2A CLU CSF1
3 inflammatory response GO:0006954 9.54 CSF1 CSF1R SERPINA3
4 hemopoiesis GO:0030097 9.4 CSF1 CSF1R
5 osteoclast differentiation GO:0030316 9.32 CSF1 CSF1R
6 macrophage differentiation GO:0030225 9.26 CSF1 CSF1R
7 negative regulation of phosphorylation GO:0042326 9.16 CDKN2A CDKN2B
8 obsolete negative regulation of cyclin-dependent protein serine/threonine kinase activity involved in G1/S transition of mitotic cell cycle GO:0031658 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 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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