MCID: TNS001
MIFTS: 36

Tenosynovial Giant Cell Tumor

Categories: 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 72
Giant Cell Tumor of Tendon Sheath 12
Giant Cell Tumor of Tenosynovium 12

Classifications:



External Ids:

Disease Ontology 12 DOID:314
ICD9CM 35 727.02
MeSH 44 D000070779
NCIt 50 C3402
UMLS 72 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 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, breast and brain, and related phenotype is no phenotypic analysis.

Wikipedia : 75 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 84)
# Related Disease Score Top Affiliating Genes
1 pigmented villonodular synovitis 33.3 CSF1R CSF1
2 giant cell tumor 32.0 SERPINA3 CSF1
3 synovitis 10.8
4 villonodular synovitis 10.8
5 tenosynovitis 10.4
6 malignant giant cell tumor 10.4
7 nodular tenosynovitis 10.4
8 hemangioma 10.3
9 chondrosarcoma 10.3
10 chondroblastoma 10.3
11 chromosomal triplication 10.3
12 rare soft tissue tumor 10.3
13 thyroid carcinoma 10.3
14 histiocytic sarcoma 10.3 CSF1 CDKN2A
15 lipomatosis, multiple 10.2
16 pleomorphic lipoma 10.2
17 chondroma 10.2
18 sclerosing hemangioma 10.2
19 neurofibromatosis, type iv, of riccardi 10.2
20 noonan syndrome 1 10.2
21 fibroma 10.2
22 hemosiderosis 10.2
23 giant cell reparative granuloma 10.2
24 pseudo-turner syndrome 10.2
25 atypical neurofibroma 10.2 CDKN2B CDKN2A
26 ewing's family of tumors 10.2 CDKN2B CDKN2A
27 carpal tunnel syndrome 10.1
28 triiodothyronine receptor auxiliary protein 10.1
29 pulmonary alveolar microlithiasis 10.1
30 hypophosphatemia 10.1
31 desmoid tumor 10.1
32 osteomalacia 10.1
33 glomus tumor 10.1
34 extraskeletal chondroma 10.1
35 pseudosarcomatous fibromatosis 10.1
36 fasciitis 10.1
37 periostitis 10.1
38 enchondroma 10.1
39 oncogenic osteomalacia 10.1
40 pyogenic granuloma 10.1
41 paresthesia 10.1
42 skin melanoma 10.1 CDKN2B CDKN2A
43 polykaryocytosis inducer 10.1
44 sarcoidosis 1 10.1
45 thrombophilia due to thrombin defect 10.1
46 tuberous sclerosis 1 10.1
47 cryptorchidism, unilateral or bilateral 10.1
48 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.1
49 sarcoma, synovial 10.1
50 aneurysmal bone cysts 10.1

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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 no phenotypic analysis MP:0003012 8.92 CDKN2A CDKN2B CSF1 CSF1R

Drugs & Therapeutics for Tenosynovial Giant Cell Tumor

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

(show all 11)
# 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 Immunologic Factors Phase 2
4 Antibodies, Monoclonal Phase 2
5 Antibodies Phase 1, Phase 2
6 Immunoglobulins Phase 1, Phase 2
7
Paclitaxel Approved, Vet_approved Phase 1 33069-62-4 36314
8 Antineoplastic Agents, Phytogenic Phase 1
9 Tubulin Modulators Phase 1
10 Albumin-Bound Paclitaxel Phase 1
11 Antimitotic Agents Phase 1

Interventional clinical trials:

(show all 11)
# 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;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 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
5 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
6 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
7 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
8 Multi-center, Phase 1, Open-Label Study of DCC-3014 to Assess the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics in Patients With Advanced Tumors Recruiting NCT03069469 Phase 1 DCC-3014
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 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) 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:

41
Bone, Breast, Brain, Salivary Gland, Thyroid

Publications for Tenosynovial Giant Cell Tumor

Articles related to Tenosynovial Giant Cell Tumor:

(show top 50) (show all 166)
# Title Authors PMID Year
1
Diffuse-Type Tenosynovial Giant Cell Tumor of the Thoracic Spine: Appearance on FDG PET/CT. 38
31274627 2019
2
Histologically benign metastasizing tenosynovial giant cell tumor mimicking metastatic malignancy: A case report and review of literature. 38
31193787 2019
3
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. 38
31318811 2019
4
Intra-articular fibroma-like perivascular epithelioid tumor (PEComa) mimicking tenosynovial giant cell tumor, diffuse type. 38
30206676 2019
5
Diffuse Tenosynovial Giant Cell Tumor Around the Temporomandibular Joint: An Entity With Special Radiologic and Pathologic Features. 38
30796908 2019
6
The effects of radiosynoviorthesis in pigmented villonodular synovitis of the knee. 38
30539286 2019
7
Massively parallel sequencing of tenosynovial giant cell tumors reveals novel CSF1 fusion transcripts and novel somatic CBL mutations. 38
31107544 2019
8
Diffuse-Type Tenosynovial Giant Cell Tumor of the Shoulder Evaluated by FDG PET/CT. 38
30624274 2019
9
Tenosynovial Giant Cell Tumor of Temporomandibular Joint and Skull Base Presenting as Ear Canal Mass. 38
30955370 2019
10
Desmoplastic fibroblastoma mimicking tenosynovial giant cell tumor encasing a tendon of the foot. 38
30229348 2019
11
Diffuse-Type Tenosynovial Giant Cell Tumor of the Thoracic Spine: Appearance on FDG PET/CT. 38
30889006 2019
12
A phase I study of pexidartinib, a colony-stimulating factor 1 receptor inhibitor, in Asian patients with advanced solid tumors. 38
30825104 2019
13
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. 38
30738433 2019
14
Giant Cell Poor Extra-Articular Diffuse-Type Tenosynovial Giant Cell Tumor With Extensive Desmin Expression: A Potential Diagnostic Pitfall With Cytogenetic Confirmation. 38
30019981 2019
15
The measurement of physical functioning among patients with Tenosynovial Giant Cell Tumor (TGCT) using the Patient-Reported Outcomes Measurement Information System (PROMIS). 38
30714073 2019
16
Malignant Tenosynovial Giant Cell Tumor: The True "Synovial Sarcoma?" A Clinicopathologic, Immunohistochemical, and Molecular Cytogenetic Study of 10 Cases, Supporting Origin from Synoviocytes. 38
30206409 2019
17
Imaging features of phosphaturic mesenchymal tumors. 38
29987349 2019
18
Tenosynovial giant cell tumor of the cervical spine: a case report. 38
31240119 2019
19
Significance of incidental intra-articular and peri-articular FDG avid foci on PET/CT. 38
29665710 2019
20
A case report of vanishing bile duct syndrome after exposure to pexidartinib (PLX3397) and paclitaxel. 38
31240240 2019
21
Extra-articular tenosynovial giant cell tumor of diffuse type in the temporal area with brain parenchymal invasion: a case report. 38
29334779 2018
22
Clinical characteristics and treatment outcomes in six cases of malignant tenosynovial giant cell tumor: initial experience of molecularly targeted therapy. 38
30594158 2018
23
Concomitant Spondyloarthritis and Tenosynovial Giant Cell Tumor in Pigmented Villonodular Synovitis Challenging Cases. 38
30601200 2018
24
[Histopathological classification principles of rheumatic joint diseases : Contribution of pathology to the diagnosis]. 38
30255358 2018
25
Distinct extra-articular invasion patterns of diffuse pigmented villonodular synovitis/tenosynovial giant cell tumor in the knee joints. 38
29637236 2018
26
Tenosynovial Giant Cell Tumor of the Clinoid: Rare Condition. 38
30026165 2018
27
Tenosynovial Giant Cell Tumors in Children: A Similar Entity Compared With Adults. 38
29494352 2018
28
Chondroid Tenosynovial Giant Cell Tumor of Temporomandibular Joint. 38
30693256 2018
29
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. 38
29663145 2018
30
Hot shoulder PET/CT lesion: Unusual presentation of tenosynovial giant cell tumor. 38
29988775 2018
31
[CT imaging features of diffuse tenosynovial giant cell tumor of temporomandibular joint]. 38
29984929 2018
32
BILATERAL LOCALIZED PIGMENTED VILLONODULAR SYNOVITIS OF THE KNEE: CASE REPORT AND REVIEW. 38
30038543 2018
33
Intraarticular calcifying aponeurotic fibroma of the wrist: mimicking gout or calcium pyrophosphate dihydrate deposition disease. 38
29243144 2018
34
Bone Involvement Mimicking an Aggressive Bone Lesion in a Diffuse-type Tenosynovial Giant Cell Tumor in the Thoracic Vertebral Lamina: A Case Report. 38
30584508 2018
35
[Diffuse-type tenosynovial giant cell tumor of the gluteus maximus muscle: a case report]. 38
29772865 2018
36
Burden of Illness Associated with Tenosynovial Giant Cell Tumors. 38
29580718 2018
37
Pitfalls in the cytological diagnosis of tenosynovial giant cell tumor: An illustration of eight discordant cases. 38
28941190 2018
38
Clusterin in Neuroendocrine Epithelial Neoplasms: Absence of Expression in a Well-differentiated Tumor Suggests a Jejunoileal Origin. 38
29420353 2018
39
The Patient Perspective on the Impact of Tenosynovial Giant Cell Tumors on Daily Living: Crowdsourcing Study on Physical Function and Quality of Life. 38
29475829 2018
40
Pigmented villonodular synovitis. 38
30745604 2018
41
Locally Aggressive Connective Tissue Tumors. 38
29220303 2018
42
Articular abnormalities that may mimic arthritis. 38
30451404 2018
43
Tenosynovial Giant Cell Tumor in the Midfoot Treated With Femoral Head Allograft Reconstruction. 38
28864387 2018
44
Tenosynovial giant cell tumor: case report of a patient effectively treated with pexidartinib (PLX3397) and review of the literature. 38
30002809 2018
45
Tenosynovial Giant Cell Tumor: Incidence, Prevalence, Patient Characteristics, and Recurrence. A Registry-based Cohort Study in Denmark. 38
28765257 2017
46
Chondroid Tenosynovial Giant Cell Tumor of the Temporomandibular Joint: A Rare Case Report. 38
29215692 2017
47
Fine needle aspiration cytology diagnosis of metastatic malignant diffuse type tenosynovial giant cell tumor. 38
28701836 2017
48
Prognosis of Advanced Tenosynovial Giant Cell Tumor of the Knee Diagnosed During Total Knee Arthroplasty. 38
28161138 2017
49
Imaging features for diffuse-type tenosynovial giant cell tumor of the temporomandibular joint: A case report. 38
28658169 2017
50
Malignant tenosynovial giant cell tumor with CDKN2A/B genomic alteration: a histological, immunohistochemical, and molecular study. 38
27989786 2017

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 platelet alpha granule lumen GO:0031093 9.16 SERPINA3 CLU
2 collagen-containing extracellular matrix GO:0062023 9.13 SERPINA3 COL6A3 CLU
3 CSF1-CSF1R complex GO:1990682 8.62 CSF1R CSF1

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

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

# Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase inhibitor activity GO:0004867 8.96 SERPINA3 COL6A3
2 cyclin-dependent protein serine/threonine kinase inhibitor activity GO:0004861 8.62 CDKN2B CDKN2A

Sources for Tenosynovial Giant Cell Tumor

3 CDC
7 CNVD
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10 dbSNP
11 DGIdb
17 EFO
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28 GO
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30 HGMD
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32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
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44 MeSH
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49 NCI
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62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
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73 UMLS via Orphanet
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