MCID: TNS001
MIFTS: 40

Tenosynovial Giant Cell Tumor

Categories: Bone diseases, Cancer diseases, Muscle diseases

Aliases & Classifications for Tenosynovial Giant Cell Tumor

MalaCards integrated aliases for Tenosynovial Giant Cell Tumor:

Name: Tenosynovial Giant Cell Tumor 12 15 17
Fibrous Histiocytoma of Tendon Sheath 12 71
Giant Cell Tumour of Tendon Sheath 12
Giant Cell Tumor of Tendon Sheath 12
Giant Cell Tumour of Tenosynovium 12
Giant Cell Tumor of Tenosynovium 12
Tenosynovial Giant Cell Tumour 12

Classifications:



External Ids:

Disease Ontology 12 DOID:314
ICD9CM 34 727.02
MeSH 43 D000070779
NCIt 49 C3402
SNOMED-CT 67 310605004
UMLS 71 C1318543

Summaries for Tenosynovial Giant Cell Tumor

MalaCards based summary : Tenosynovial Giant Cell Tumor, also known as fibrous histiocytoma of tendon sheath, is related to pigmented villonodular synovitis and malignant giant cell tumor of the tendon sheath. An important gene associated with Tenosynovial Giant Cell Tumor is CSF1 (Colony Stimulating Factor 1), and among its related pathways/superpathways are Chemokine Superfamily: Human/Mouse Ligand-Receptor Interactions and Hematopoietic cell lineage. The drugs Sargramostim and Grapefruit Seed Extract have been mentioned in the context of this disorder. Affiliated tissues include bone, breast and brain, and related phenotypes are cellular and homeostasis/metabolism

Wikipedia : 74 Pigmented villonodular synovitis (PVNS) , also known as intra-articular giant-cell tumor of the tendon... more...

Related Diseases for Tenosynovial Giant Cell Tumor

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

(show top 50) (show all 237)
# Related Disease Score Top Affiliating Genes
1 pigmented villonodular synovitis 33.5 SERPINA3 CSF1R CSF1 CD68
2 malignant giant cell tumor of the tendon sheath 32.7 CSF1R CSF1 COL6A3 CD68
3 villonodular synovitis 32.0 CSF1 CD68
4 benign giant cell tumor 31.2 USP6 SERPINA3 CSF1R CSF1 CD68
5 nodular tenosynovitis 30.9 SERPINA3 AKTIP
6 giant cell reparative granuloma 30.6 USP6 SERPINA3 CD68
7 multicentric reticulohistiocytosis 30.6 SERPINA3 CSF1 CD68
8 pleomorphic lipoma 30.5 SERPINA3 CD68 CD34
9 lipomatosis, multiple 30.3 SERPINA3 KIT CD34
10 bone giant cell tumor 30.2 USP6 SERPINA3 CSF1 CD68
11 desmoid tumor 30.1 KIT CDKN2A CD34
12 fibroma 30.1 USP6 SERPINA3 CD68 CD34
13 muscle cancer 30.0 SERPINA3 KIT CD34
14 sarcoma, synovial 29.9 SERPINA3 KIT CD34
15 malignant peripheral nerve sheath tumor 29.8 KIT CDKN2A CD34
16 fibrous histiocytoma 29.4 SERPINA3 KIT CD68 CD34 CD163
17 pseudosarcomatous fibromatosis 29.2 USP6 SERPINA3 CD68 CD34 AKTIP
18 plica syndrome 10.9
19 synovitis 10.9
20 bone giant cell sarcoma 10.4 CSF1 CD163
21 verruciform xanthoma of skin 10.4 SERPINA3 CD68
22 congenital epulis 10.4 SERPINA3 CD68
23 epulis 10.4 SERPINA3 CD68
24 tenosynovitis 10.4
25 lymphohistiocytoid mesothelioma 10.4 CDKN2A CD68
26 xanthogranulomatous pyelonephritis 10.4 SERPINA3 CD68
27 malignant inflammatory fibrous histiocytoma 10.4 SERPINA3 CD68
28 pancreas sarcoma 10.4 SERPINA3 CD68
29 primary cutaneous b-cell lymphoma 10.4 CDKN2B CDKN2A
30 xanthogranulomatous cholecystitis 10.4 SERPINA3 CD68
31 interdigitating dendritic cell sarcoma 10.4 SERPINA3 CD68
32 malignant giant cell tumor 10.4
33 hemosiderosis 10.4
34 chondroblastoma 10.4
35 spondylarthropathy 10.4 CSF1 CD163
36 cervical adenoid cystic carcinoma 10.3 KIT CDKN2A
37 langerhans cell sarcoma 10.3 CD68 CD163
38 melanoma-astrocytoma syndrome 10.3 CDKN2B CDKN2A
39 necrobiosis lipoidica 10.3 CD68 CD163
40 balloon cell malignant melanoma 10.3 CD68 CD163
41 hemangioma 10.3
42 laryngeal small cell carcinoma 10.3 KIT CDKN2A
43 chondrosarcoma 10.3
44 chromosomal triplication 10.3
45 rare soft tissue tumor 10.3
46 thyroid carcinoma 10.3
47 cutaneous solitary mastocytoma 10.3 SERPINA3 KIT
48 axillary lipoma 10.3 KIT CD68
49 type c thymoma 10.3 SERPINA3 KIT
50 small intestinal sarcoma 10.3 KIT CD68

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



Diseases related to Tenosynovial Giant Cell Tumor

Symptoms & Phenotypes for Tenosynovial Giant Cell Tumor

MGI Mouse Phenotypes related to Tenosynovial Giant Cell Tumor:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.17 AKTIP CCR2 CD163 CD34 CDKN2A CDKN2B
2 homeostasis/metabolism MP:0005376 10.15 AKTIP CCR2 CD163 CD34 CDKN2A CDKN2B
3 hematopoietic system MP:0005397 10.1 CCR2 CD163 CD34 CD68 CDKN2A CDKN2B
4 immune system MP:0005387 10.1 AKTIP CCR2 CD163 CD34 CD68 CDKN2A
5 integument MP:0010771 9.86 AKTIP CD34 CDKN2A CDKN2B CSF1 CSF1R
6 neoplasm MP:0002006 9.63 AKTIP CCR2 CD34 CDKN2A CDKN2B KIT
7 no phenotypic analysis MP:0003012 9.56 CCR2 CD68 CDKN2A CDKN2B CSF1 CSF1R
8 skeleton MP:0005390 9.23 AKTIP CCR2 CD68 CDKN2A COL6A3 CSF1

Drugs & Therapeutics for Tenosynovial Giant Cell Tumor

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

(show all 8)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sargramostim Approved, Investigational Phase 3 83869-56-1, 123774-72-1
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 Albumin-Bound Paclitaxel Phase 1
7 Tubulin Modulators Phase 1
8 Antimitotic Agents Phase 1

Interventional clinical trials:

(show all 12)
# Name Status NCT ID Phase Drugs
1 A Double-blind, Randomized, Placebo-controlled Phase 3 Study of Orally Administered PLX3397 in Subjects With Pigmented Villonodular Synovitis or Giant Cell Tumor of the Tendon Sheath Active, not recruiting NCT02371369 Phase 3 Pexidartinib;Placebo
2 A Phase II Randomized, Double -Blind, Placebo Controlled Study to Assess Safety, Tolerability and Effect on Tumor Size of MCS110 in Patients With Pigmented Villonodular Synovitis (PVNS) Completed NCT01643850 Phase 2 MCS110;Placebo
3 Phase II Study of Nilotinib Efficacy in Pigmented Villo-Nodular Synovitis/ Tenosynovial Giant Cell Tumour (PVNS/TGCT) Completed NCT01261429 Phase 2 Tasigna
4 A Multicenter Phase 1/2, Open-Label Study of DCC-3014 to Assess the Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics in Patients With Advanced Tumors and Tenosynovial Giant Cell Tumor Recruiting NCT03069469 Phase 1, Phase 2 DCC-3014
5 A PHASE 1B/2 OPEN-LABEL STUDY TO EVALUATE SAFETY, CLINICAL ACTIVITY, PHARMACOKINETICS AND PHARMACODYNAMICS OF AVELUMAB (MSB0010718C) IN COMBINATION WITH OTHER CANCER IMMUNOTHERAPIES IN PATIENTS WITH ADVANCED MALIGNANCIES Recruiting NCT02554812 Phase 2 Avelumab;Utomilumab;PF-04518600;PD 0360324;CMP-001
6 A Phase 1/2 Study of Cabiralizumab, an Anti-CSF1 Receptor Antibody, in Patients With Pigmented Villonodular Synovitis (PVNS)/ Diffuse Type Tenosynovial Giant Cell Tumor (Dt-TGCT) Active, not recruiting NCT02471716 Phase 1, Phase 2
7 A Multi-Center Single Agent Phase II Study of the Efficacy of 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
8 Open-Label, Multicenter, Dose Escalation Phase Ia/Ib Study With Expansion Phase to Evaluate Safety, Pharmacokinetics and Activity of RO5509554, Administered as an Intravenous Infusion as Monotherapy and in Combination With Paclitaxel in Patients With Advanced Solid Tumors Completed NCT01494688 Phase 1 Paclitaxel;RO5509554
9 A Phase 1 Study to Assess Safety, Pharmacokinetics, and Pharmacodynamics of PLX73086 as a Single Agent in Subjects With Advanced Solid Tumors and in Subjects With Locally Advanced or Refractory Tenosynovial Giant Cell Tumor (TGCT) Terminated NCT02673736 Phase 1 PLX73086
10 A Phase 1 Study to Assess Safety, Pharmacokinetics, and Pharmacodynamics of PLX7486 as a Single Agent in Patients With Advanced Solid Tumors Terminated NCT01804530 Phase 1 PLX7486 TsOH
11 An Open-label, Single-dose Study to Assess the Pharmacokinetics of Pexidartinib in Subjects With Moderate Hepatic Impairment Compared to Healthy Subjects Recruiting NCT04223635 Early Phase 1 Pexidartinib
12 A Disease Registry for Patients With Tenosynovial Giant Cell Tumors (TGCT), Also Known as Pigmented Villonodular Synovitis (PVNS) and Giant Cell Tumor of the Tendon Sheath (GCT-TS) Active, not recruiting NCT02948088

Search NIH Clinical Center for Tenosynovial Giant Cell Tumor

Genetic Tests for Tenosynovial Giant Cell Tumor

Anatomical Context for Tenosynovial Giant Cell Tumor

MalaCards organs/tissues related to Tenosynovial Giant Cell Tumor:

40
Bone, Breast, Brain, Thyroid, Salivary Gland

Publications for Tenosynovial Giant Cell Tumor

Articles related to Tenosynovial Giant Cell Tumor:

(show top 50) (show all 193)
# Title Authors PMID Year
1
Management of Pigmented Villonodular Synovitis (PVNS): an Orthopedic Surgeon's Perspective. 61
32494892 2020
2
Pexidartinib for the treatment of adult symptomatic patients with tenosynovial giant cell tumors. 61
32478598 2020
3
Tenosynovial Giant Cell Tumor, Localized Type With Extensive Chondroid Metaplasia: A Case Report With Immunohistochemical and Molecular Genetic Analysis. 61
31771368 2020
4
Tenosynovial Giant Cell Tumor in the Hand: Experience with 173 Cases. 61
32312203 2020
5
PK/PD mediated dose optimization of emactuzumab, a CSF1R inhibitor, in patients with advanced solid tumors and diffuse-type tenosynovial giant cell tumor. 61
32575160 2020
6
Tenosynovial Giant Cell Tumor in an Infant. 61
32472881 2020
7
Shoulder Tumor/Tumor-Like Lesions: What to Look for. 61
32241665 2020
8
Tenosynovial giant cell tumor mimicking an ankle monoarthritis. 61
32384050 2020
9
Pexidartinib for the treatment of adult patients with symptomatic tenosynovial giant cell tumor: safety and efficacy. 61
32297819 2020
10
Tenosynovial Giant Cell Tumor of Temporomandibular Joint and Skull Base Presenting as Ear Canal Mass. 61
30955370 2020
11
Unusual manifestations of diffuse-type tenosynovial giant cell tumor in two patients: importance of radiologic-pathologic correlation. 61
31656976 2020
12
Soft Tissue Special Issue: Giant Cell-Rich Lesions of the Head and Neck Region. 61
31950466 2020
13
Uncommon tumors of temporomandibular joint: An institutional experience and review. 61
32040228 2020
14
Intraarticular Nodular Fasciitis of the Knee With MHY9-USP6 Fusion: A Case Report. 61
32089028 2020
15
A phase I study of pexidartinib, a colony-stimulating factor 1 receptor inhibitor, in Asian patients with advanced solid tumors. 61
30825104 2020
16
Monoarticular, Multifocal, Localized Tenosynovial Giant Cell Tumor of the Knee: A Case Report. 61
32044773 2020
17
Pexidartinib, a Novel Small Molecule CSF-1R Inhibitor in Use for Tenosynovial Giant Cell Tumor: A Systematic Review of Pre-Clinical and Clinical Development. 61
32440095 2020
18
Incomplete resection increases the risk of local recurrence and negatively affects functional outcome in patients with tenosynovial giant cell tumor of the hindfoot. 61
31839476 2019
19
KRAS mutant tenosynovial giant cell tumor in a pediatric patient: a case report. 61
31993359 2019
20
Massively parallel sequencing of tenosynovial giant cell tumors reveals novel CSF1 fusion transcripts and novel somatic CBL mutations. 61
31107544 2019
21
Molecular Profiling of Atypical Tenosynovial Giant Cell Tumors Reveals Novel Non-CSF1 Fusions. 61
31906059 2019
22
Risk Factors Associated With Postoperative Recurrence in Patients With Tenosynovial Giant Cell Tumor of the Hand: A Retrospective Cohort Study. 61
31524641 2019
23
Pexidartinib: First Approval. 61
31602563 2019
24
Long-term efficacy of imatinib mesylate in patients with advanced Tenosynovial Giant Cell Tumor. 61
31601938 2019
25
Tenosynovial giant cell tumor in the foot and ankle. 61
31526689 2019
26
The surgical outcome and recurrence rate of tenosynovial giant cell tumor in the elbow: a literature review. 61
31447124 2019
27
A Pregnant Woman With Multi-fragmented Giant Cell Tumor of Tendon Sheath: A Rare Anatomical Location. 61
31486673 2019
28
Diffuse-Type Tenosynovial Giant Cell Tumor of the Thoracic Spine: Appearance on FDG PET/CT. 61
31274627 2019
29
Histologically benign metastasizing tenosynovial giant cell tumor mimicking metastatic malignancy: A case report and review of literature. 61
31193787 2019
30
Surgical Treatment of Localized-Type Tenosynovial Giant Cell Tumors of Large Joints: A Study Based on a Multicenter-Pooled Database of 31 International Sarcoma Centers. 61
31318811 2019
31
Intra-articular fibroma-like perivascular epithelioid tumor (PEComa) mimicking tenosynovial giant cell tumor, diffuse type. 61
30206676 2019
32
Diffuse Tenosynovial Giant Cell Tumor Around the Temporomandibular Joint: An Entity With Special Radiologic and Pathologic Features. 61
30796908 2019
33
The effects of radiosynoviorthesis in pigmented villonodular synovitis of the knee. 61
30539286 2019
34
Diffuse-Type Tenosynovial Giant Cell Tumor of the Shoulder Evaluated by FDG PET/CT. 61
30624274 2019
35
Desmoplastic fibroblastoma mimicking tenosynovial giant cell tumor encasing a tendon of the foot. 61
30229348 2019
36
Diffuse-Type Tenosynovial Giant Cell Tumor of the Thoracic Spine: Appearance on FDG PET/CT. 61
30889006 2019
37
Randomized placebo-controlled double-blind phase II study of zaltoprofen for patients with diffuse-type and unresectable localized tenosynovial giant cell tumors: a study protocol. 61
30738433 2019
38
Malignant Tenosynovial Giant Cell Tumor: The True "Synovial Sarcoma?" A Clinicopathologic, Immunohistochemical, and Molecular Cytogenetic Study of 10 Cases, Supporting Origin from Synoviocytes. 61
30206409 2019
39
Tenosynovial giant cell tumor of the cervical spine: a case report. 61
32094332 2019
40
The measurement of physical functioning among patients with Tenosynovial Giant Cell Tumor (TGCT) using the Patient-Reported Outcomes Measurement Information System (PROMIS). 61
30714073 2019
41
Giant Cell Poor Extra-Articular Diffuse-Type Tenosynovial Giant Cell Tumor With Extensive Desmin Expression: A Potential Diagnostic Pitfall With Cytogenetic Confirmation. 61
30019981 2019
42
Imaging features of phosphaturic mesenchymal tumors. 61
29987349 2019
43
Clinical and morphological correlations and histopathology of joint damage in patients with diffuse-type tenosynovial giant cell tumor. 61
32124739 2019
44
Tenosynovial giant cell tumor of the cervical spine: a case report. 61
31240119 2019
45
Expression of PD-L1 in mononuclear cells, multinucleated cells, and foam cells in tenosynovial giant cell tumors. 61
31933896 2019
46
Significance of incidental intra-articular and peri-articular FDG avid foci on PET/CT. 61
29665710 2019
47
A case report of vanishing bile duct syndrome after exposure to pexidartinib (PLX3397) and paclitaxel. 61
31240240 2019
48
Concomitant Spondyloarthritis and Tenosynovial Giant Cell Tumor in Pigmented Villonodular Synovitis Challenging Cases. 61
30601200 2018
49
Extra-articular tenosynovial giant cell tumor of diffuse type in the temporal area with brain parenchymal invasion: a case report. 61
29334779 2018
50
Clinical characteristics and treatment outcomes in six cases of malignant tenosynovial giant cell tumor: initial experience of molecularly targeted therapy. 61
30594158 2018

Variations for Tenosynovial Giant Cell Tumor

Expression for Tenosynovial Giant Cell Tumor

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

Pathways for Tenosynovial Giant Cell Tumor

GO Terms for Tenosynovial Giant Cell Tumor

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

# Name GO ID Score Top Affiliating Genes
1 CSF1-CSF1R complex GO:1990682 8.62 CSF1R CSF1

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.8 KIT IL34 CSF1 CLU CDKN2A CD34
2 cytokine-mediated signaling pathway GO:0019221 9.77 KIT IL34 CSF1R CSF1 CCR2
3 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.7 KIT CSF1R CSF1
4 negative regulation of phosphorylation GO:0042326 9.51 CDKN2B CDKN2A
5 inflammatory response GO:0006954 9.5 SERPINA3 KIT IL34 CSF1R CSF1 CD163
6 positive regulation of macrophage differentiation GO:0045651 9.49 IL34 CSF1
7 positive regulation of monocyte differentiation GO:0045657 9.48 IL34 CSF1
8 somatic stem cell division GO:0048103 9.46 KIT CDKN2A
9 hemopoiesis GO:0030097 9.46 KIT CSF1R CD34 CCR2
10 positive regulation of macrophage proliferation GO:0120041 9.43 IL34 CSF1R
11 positive regulation of macrophage chemotaxis GO:0010759 9.43 IL34 CSF1R CSF1
12 macrophage colony-stimulating factor signaling pathway GO:0038145 9.4 CSF1R CSF1
13 microglial cell proliferation GO:0061518 8.92 IL34 CSF1R CSF1 CLU

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 9.16 CDKN2B CDKN2A
2 cytokine binding GO:0019955 9.13 KIT CSF1R CCR2
3 macrophage colony-stimulating factor receptor binding GO:0005157 8.62 IL34 CSF1

Sources for Tenosynovial Giant Cell Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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