TGCT
MCID: PGM001
MIFTS: 61

Pigmented Villonodular Synovitis (TGCT) malady

Rare diseases, Bone diseases, Muscle diseases, Cancer diseases categories
Download this MalaCard

Summaries for Pigmented Villonodular Synovitis

About this section


Fully expand this MalaCard
NIH Rare Diseases:42 Pigmented villonodular synovitis is a disease in which the tissue lining a joint in the body begins to grow abnormally.  there are two types of pigmented villonodular synovitis: the nodular form (where the abnormal tissue growth forms a single bump) and the diffuse form (where the entire lining of the joint grows unusually).  the additional tissue in the joint can cause pain, limit movements or cause the joint to lock, and in some cases, can destroy the normal joint structure.  the knee is most commonly affected by this condition, though it can occur in other joints such as the hip, shoulder, elbow, ankle, or wrist.  the average age of diagnosis for this condition is 35 years. last updated: 11/28/2011

MalaCards based summary: Pigmented Villonodular Synovitis, also known as tenosynovial giant cell tumor, is related to synovitis and giant cell tumor. An important gene associated with Pigmented Villonodular Synovitis is IBSP (integrin-binding sialoprotein), and among its related pathways are Syndecan-1-mediated signaling events and Osteopontin Signaling. The compounds pyridinoline and ibandronate have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and testes, and related mouse phenotypes are craniofacial and limbs/digits/tail.

Wikipedia:65 Pigmented villonodular synovitis (PVNS) is a joint disease characterized by inflammation and overgrowth... more...

Aliases & Classifications for Pigmented Villonodular Synovitis

About this section
Sources:
8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 48Orphanet, 62UMLS, 44Novoseek, 57SNOMED-CT, 39NCIt, 34MeSH, 35MESH via Orphanet, 63UMLS via Orphanet
See all sources

Pigmented Villonodular Synovitis, Aliases & Descriptions:

Name: Pigmented Villonodular Synovitis 8 42 10 48 62
Tenosynovial Giant Cell Tumor 48 62
Tgct 48 62
Localized Pigmented Villonodular Synovitis 42
Diffuse Pigmented Villonodular Synovitis 42
Diffuse Giant Cell Tumor of Tenosynovium 8
Fibrous Histiocytoma of Tendon Sheath 62
 
Synovitis Pigmented Villonodular 44
Tenosynovial Giant Cell Tumors 42
Diffuse-Type Giant Cell Tumor 48
Villous Tenosynovitis 8
Diffuse-Type Gct 48
Tsgct 48


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases, Cancer diseases
Anatomical: Bone diseases, Muscle diseases


External Ids:

Disease Ontology8 DOID:2702
NCIt39 C3401
MeSH34 D013586
MESH via Orphanet35 D013586
UMLS via Orphanet63 C0039106

Related Diseases for Pigmented Villonodular Synovitis

About this section

Diseases related to Pigmented Villonodular Synovitis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 132)
idRelated DiseaseScoreTop Affiliating Genes
1synovitis32.8CD68, MMP1, TNFRSF11B, TNFSF11
2giant cell tumor31.6TNFSF11, CSF1R, CSF1
3osteonecrosis31.5BGLAP
4ankylosing spondylitis30.9TNFRSF11B, TNFSF11, BGLAP
5arthritis30.1BGLAP, TNFSF11, TNFRSF11B, MMP9, MMP1, ACP5
6rheumatoid arthritis29.9TNFSF11, IBSP, BGLAP, SPP1, TNFRSF11B, MMP9
7osteoarthritis28.8MMP1, MMP9, PTHLH, TNFRSF11B, TNFSF11, IBSP
8melanoma28.5SPP1, IBSP, TNFSF11, TNFRSF11B, MMP9, MMP1
9villonodular synovitis11.7
10tenosynovial giant cell tumor10.8
11multicentric reticulohistiocytosis10.6CD68
12histiocytosis10.6CD68
13malignant giant cell tumor10.6
14neuropathy10.6
15camurati-engelmann disease10.5BGLAP
16hemarthrosis10.5
17carpal tunnel syndrome10.5
18tuberculosis10.5
19synovial chondromatosis10.5
20cherubism10.5TNFSF11
21periapical granuloma10.4TNFSF11
22pseudohypoparathyroidism type 1b10.4PTHLH, BGLAP
23male germ cell tumor, somatic10.4
24osteopetrosis autosomal dominant type 210.4BGLAP, TNFSF11
25paget disease, juvenile10.4TNFSF11, TNFRSF11B
26polyostotic osteolytic dysplasia, hereditary expansile10.4TNFSF11, TNFRSF11B
27fibrous dysplasia10.4IBSP, BGLAP
28pseudohypoparathyroidism10.4PTHLH, BGLAP
29osteosclerosis10.4TNFRSF11B, TNFSF11
30osteomyelitis10.4IBSP, TNFSF11, ACP5
31dentin dysplasia10.4SPP1, IBSP
32nephrolithiasis10.3IBSP, SPP1
33osteofibrous dysplasia10.3BGLAP, SPP1
34renal osteodystrophy10.3BGLAP, TNFRSF11B, ACP5
35dentinogenesis imperfecta10.3SPP1, IBSP
36hemangioma10.3
37systemic lupus erythematosus10.3
38malignant giant cell tumor of the tendon sheath10.3
39nodular tenosynovitis10.3
40chondroblastoma10.3
41ulnar neuropathy10.3
42peroneal neuropathy10.3
43noonan syndrome10.3
44bone giant cell tumor10.3
45cervicitis10.3
46conjunctivitis10.3
47lipoma10.3
48lupus erythematosus10.3
49paraplegia10.3
50sarcoma10.3

Graphical network of the top 20 diseases related to Pigmented Villonodular Synovitis:



Diseases related to pigmented villonodular synovitis

Symptoms for Pigmented Villonodular Synovitis

About this section

Drugs & Therapeutics for Pigmented Villonodular Synovitis

About this section

Drug clinical trials:

Search ClinicalTrials for Pigmented Villonodular Synovitis

Search NIH Clinical Center for Pigmented Villonodular Synovitis

Genetic Tests for Pigmented Villonodular Synovitis

About this section

Anatomical Context for Pigmented Villonodular Synovitis

About this section

MalaCards organs/tissues related to Pigmented Villonodular Synovitis:

32
Bone, Lung, Testes

Animal Models for Pigmented Villonodular Synovitis or affiliated genes

About this section

Publications for Pigmented Villonodular Synovitis

About this section

Articles related to Pigmented Villonodular Synovitis:

(show top 50)    (show all 508)
idTitleAuthorsYear
1
Transtrochanteric rotational osteotomy combined with intra-articular procedures for pigmented villonodular synovitis of the hip. (24687213)
2014
2
Unusual cases of pigmented villonodular synovitis after arthroplasty. (24955198)
2014
3
Pigmented villonodular synovitis diagnostic delay due to coexistence with ankylosing spondylitis. (24642231)
2014
4
Low-dose external beam radiotherapy as a postoperative treatment for patients with diffuse pigmented villonodular synovitis of the knee: 4 recurrences in 23 patients followed for mean 9 years. (22489888)
2012
5
Hip joint pigmented villonodular synovitis in a young girl: a case report. (22433958)
2012
6
Pigmented villonodular synovitis of the ankle presenting as a persisting ankle effusion. (22764668)
2012
7
The management of diffuse-type giant cell tumour (pigmented villonodular synovitis) and giant cell tumour of tendon sheath (nodular tenosynovitis). (22733940)
2012
8
One step open synovectomy without adjuvant therapy for diffuse pigmented villonodular synovitis of the knee in a soccer player. (23738243)
2011
9
Destructive synovial hemangioma of the hip resembling pigmented villonodular synovitis. (20570091)
2011
10
Pigmented villonodular synovitis of the temporomandibular joint: case report and review of the literature. (21310347)
2011
11
Radiation therapy for treatment of pigmented villonodular synovitis: results of a national patterns of care study. (20488628)
2010
12
Arthroscopic treatment of pigmented villonodular synovitis involving bilateral shoulders. (20806759)
2010
13
Tenosynovial giant cell tumor and pigmented villonodular synovitis: a proposal for unification of these clinically distinct but histologically and genetically identical lesions. (17225151)
2007
14
Pigmented villonodular synovitis of the elbow in a 6-year-old girl: a case report. (17429130)
2007
15
Radial and ulnar neuropathy due to pigmented villonodular synovitis of the elbow. (17126239)
2006
16
Pigmented villonodular synovitis. (16311109)
2006
17
Similarities between giant cell tumor of bone, giant cell tumor of tendon sheath, and pigmented villonodular synovitis concerning ultrastructural cytochemical features of multinucleated giant cells and mononuclear stromal cells. (16825116)
2006
18
Clustered localized pigmented villonodular synovitis. (15944639)
2005
19
Pigmented villonodular synovitis of the knee: disease of the popliteus tendon and posterolateral compartment. (16012505)
2005
20
Pigmented villonodular synovitis: a disease in evolution. (15290750)
2004
21
Incidental detection of pigmented villonodular synovitis on FDG PET. (12897655)
2003
22
Pigmented villonodular synovitis of the anserine bursa. (12817733)
2003
23
Pigmented villonodular synovitis of the knee presenting as sciatica. (12943290)
2003
24
Tuberculosis of the hip as the presenting sign of HIV and simulating pigmented villonodular synovitis. (12107577)
2002
25
Langerhans cell granulomatosis manifested as pigmented villonodular synovitis. (12132822)
2001
26
Arthroscopic treatment of pigmented villonodular synovitis of the shoulder. (11154374)
2001
27
Giant cell tumor of tendon sheath, tenosynovial giant cell tumor, and pigmented villonodular synovitis: defining the presentation, surgical therapy and recurrence. (10671695)
2000
28
Dynamic MR-imaging in the diagnosis of pigmented villonodular synovitis of the knee. (11093605)
2000
29
Magnetic resonance imaging evaluation and arthroscopic resection of localized pigmented villonodular synovitis of the knee. (10791586)
2000
30
Lipoma induced by pigmented villonodular synovitis of the ankle. (10359296)
1999
31
An unusual case of pigmented villonodular synovitis of the spine: benign aggressive and/or malignant? (10371292)
1999
32
Pigmented villonodular synovitis of the foot and ankle: a review of six cases. (10353773)
1999
33
Pigmented villonodular synovitis and related lesions: the spectrum of imaging findings. (9888766)
1999
34
Cytokine and matrix metalloproteinase expression in pigmented villonodular synovitis may mediate bone and cartilage destruction. (9807705)
1998
35
Diffuse pigmented villonodular synovitis of the knee associated with chronic ACL deficiency. (9606698)
1998
36
Total knee arthroplasty in patients who have pigmented villonodular synovitis. (9469312)
1998
37
Articular pigmented villonodular synovitis of the MP joint of the hand. (8543887)
1995
38
Diagnostic features of diffuse pigmented villonodular synovitis of the knee. (8118978)
1994
39
Pigmented villonodular synovitis of the temporomandibular joint. (8228609)
1993
40
Pigmented villonodular synovitis of the knee demonstrated on bone scan. Correlation with US, CT, and MRI. (1424384)
1992
41
Evaluation of the hip with pigmented villonodular synovitis. A case report. (1735210)
1992
42
Case report 578: Pigmented villonodular synovitis of the shoulder. (2321043)
1990
43
Pigmented villonodular synovitis in a vertebra. A case report. (2365726)
1990
44
Diffuse pigmented villonodular synovitis of the temporomandibular joint diagnosed by fine-needle aspiration cytology. (2791838)
1989
45
Case report 563: Pigmented villonodular synovitis (PVNS) of knee. (2814557)
1989
46
Pigmented villonodular synovitis of the knee: results from 13 cases. (2706054)
1989
47
A case of aggressive pigmented villonodular synovitis. (3421087)
1988
48
Lipid microspherules in synovial fluid of patients with pigmented villonodular synovitis. (3190786)
1988
49
Pigmented villonodular synovitis of the hip--association with osteochondromatosis. (3440329)
1987
50
Pigmented villonodular synovitis of the temporomandibular joint: a case report. (10994414)
1982

Variations for Pigmented Villonodular Synovitis

About this section

Expression for genes affiliated with Pigmented Villonodular Synovitis

About this section
Expression patterns in normal tissues for genes affiliated with Pigmented Villonodular Synovitis

Search GEO for disease gene expression data for Pigmented Villonodular Synovitis.

Pathways for genes affiliated with Pigmented Villonodular Synovitis

About this section

Pathways related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

(show all 23)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.9MMP9, MMP1
29.9SPP1, MMP9
3
Show member pathways
RANKL/RANK Signaling Pathway37
Apoptosis and survival APRIL and BAFF signaling60
9.8ACP5, TNFRSF11B, TNFSF11
49.7CD68, CSF1
5
Show member pathways
Apoptosis and survival TNFR1 signaling pathway60
TWEAK Signaling Pathway37
Apoptosis Modulation by HSP7037
HIV-1 Nef- Negative effector of Fas and TNF-alpha37
9.7MMP9, TNFRSF11B, TNFSF11
69.7MMP1, MMP9, BGLAP
79.7MMP9, PTHLH, SPP1
89.6SPP1, BGLAP, MMP9
99.5CSF1, CSF1R, SPP1
109.5SPP1, TNFSF11, TNFRSF11B, ACP5
119.5TNFRSF11B, TNFSF11, BGLAP, SPP1
129.5CSF1R, CSF1, PDGFRB
139.5PDGFRB, CSF1, CSF1R
149.4IBSP, TNFSF11, TNFRSF11B, PDGFRB
15
Show member pathways
9.4SPP1, IBSP, MMP9, MMP1
169.3LAPTM5, ACP5, CD68
179.3PTHLH, TNFSF11, IBSP, BGLAP, SPP1
189.2PDGFRB, CSF1R, MMP1, MMP9
199.1TNFSF11, TNFRSF11B, ACP5, CSF1R, CSF1
209.0SPP1, IBSP, CSF1R, CSF1, PDGFRB
21
Show member pathways
9.0TNFSF11, TNFRSF11B, CSF1R, CSF1, PDGFRB
22
Show member pathways
8.9PDGFRB, CSF1, CSF1R, TNFSF11, SPP1
238.6CSF1, ACP5, MMP1, TNFSF11, ATP6V1B2

Compounds for genes affiliated with Pigmented Villonodular Synovitis

About this section

Compounds related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

(show top 50)    (show all 174)
idCompoundScoreTop Affiliating Genes
1pyridinoline4410.6TNFRSF11B
2ibandronate44 1110.8TNFRSF11B, IBSP, BGLAP, TNFSF11
3deoxypyridinoline449.7TNFSF11, IBSP, ACP5, BGLAP, TNFRSF11B
4pamidronate44 50 1111.6BGLAP, TNFSF11, TNFRSF11B, PTHLH, MMP1
5tartrate449.6CSF1, PTHLH, TNFSF11, BGLAP, ACP5
6alendronate44 50 1111.6PTHLH, TNFRSF11B, TNFSF11, MMP1, BGLAP
7vitamin k2449.5BGLAP, TNFSF11, TNFRSF11B, CSF1
8clodronate44 50 1111.5MMP1, TNFSF11, TNFRSF11B, PTHLH, BGLAP, ACP5
9titanium449.5SPP1, IBSP, ACP5, TNFRSF11B, TNFSF11, BGLAP
1025-hydroxyvitamin d449.4PTHLH, TNFSF11, TNFRSF11B, ACP5, BGLAP, SPP1
11phosphorus449.4BGLAP, PTHLH, SPP1, TNFSF11, ACP5, TNFRSF11B
12infliximab44 50 1111.4MMP1, TNFRSF11B, BGLAP, SPP1, TNFSF11
13hydroxyapatite449.2IBSP, TNFSF11, BGLAP, SPP1, TNFRSF11B, PTHLH
14hydroxyproline44 24 1111.2ACP5, MMP9, PTHLH, TNFRSF11B, IBSP, MMP1
15zoledronic acid449.2BGLAP, IBSP, TNFSF11, TNFRSF11B, PTHLH, MMP9
16azathioprine44 2 50 1112.1PCNA, PTHLH, TNFRSF11B, BGLAP, TNFSF11, ACP5
17geranylgeranyl pyrophosphate449.1TNFRSF11B, MMP9, MMP1, TNFSF11
18celecoxib44 61 28 50 24 1113.9TNFSF11, TNFRSF11B, MMP9, MMP1, PCNA, CSF1
19prostaglandin448.8PTHLH, MMP9, MMP1, CSF1, TNFRSF11B, TNFSF11
20tgf beta1448.8PTHLH, SPP1, TNFRSF11B, MMP1, CSF1, PDGFRB
21matrigel448.8IBSP, SPP1, PTHLH, MMP9, PDGFRB, MMP1
22rantes448.7MMP1, MMP9, TNFRSF11B, TNFSF11, CSF1, ACP5
23ns 39844 619.7BGLAP, TNFSF11, TNFRSF11B, MMP9, PCNA, MMP1
24pd 98,059448.7CSF1, CSF1R, MMP9, TNFSF11, IBSP, MMP1
25estradiol44 24 1110.7IBSP, ACP5, MMP1, TNFRSF11B, TNFSF11, PTHLH
261,25 dihydroxy vitamin d3448.6CSF1, CSF1R, ACP5, PTHLH, TNFRSF11B, TNFSF11
27nicotine44 28 50 1111.6MMP1, PTHLH, CSF1, SPP1, PCNA, MMP9
28indomethacin44 28 61 1111.6SPP1, TNFSF11, PTHLH, CSF1, TNFRSF11B, PCNA
29vitamin d448.6BGLAP, SPP1, CSF1R, TNFSF11, IBSP, PTHLH
30ascorbic acid44 249.5SPP1, CSF1, BGLAP, IBSP, TNFRSF11B, MMP1
31calcitriol44 61 24 1111.5TNFSF11, BGLAP, SPP1, TNFRSF11B, PTHLH, MMP1
32cyclosporin a44 28 6110.5TNFRSF11B, BGLAP, SPP1, TNFSF11, MMP9, MMP1
33cycloheximide448.4SPP1, MMP9, TNFRSF11B, TNFSF11, MMP1, IBSP
34procollagen448.4SPP1, BGLAP, IBSP, TNFSF11, TNFRSF11B, PCNA
35heparin44 28 24 1111.3PTHLH, BGLAP, TNFSF11, IBSP, SPP1, TNFRSF11B
36genistein44 28 61 2 24 1113.2PCNA, MMP9, SPP1, PDGFRB, TNFSF11, MMP1
37phosphatidylinositol448.2IBSP, SPP1, TNFRSF11B, PTHLH, TNFSF11, CSF1R
38h2o2448.1TNFSF11, PDGFRB, MMP1, BGLAP, SPP1, CSF1
39progesterone44 28 61 24 1112.1SPP1, TNFRSF11B, PTHLH, MMP1, CSF1R, CSF1
40pge2448.0TNFSF11, TNFRSF11B, PTHLH, MMP9, MMP1, ACP5
41creatinine447.8CSF1, PCNA, ACP5, MMP1, MMP9, PTHLH
42testosterone44 61 24 1110.8CSF1, TNFRSF11B, TNFSF11, PTHLH, MMP9, BGLAP
43tyrosine447.7CSF1, CSF1R, ACP5, MMP9, PTHLH, TNFRSF11B
44serine447.7TNFRSF11B, IBSP, BGLAP, SPP1, PTHLH, MMP9
45retinoic acid44 248.7CSF1R, PDGFRB, CSF1, PCNA, TNFRSF11B, MMP1
46dexamethasone44 50 28 1110.5CSF1, CSF1R, PCNA, ACP5, MMP1, MMP9
47vegf447.3IBSP, BGLAP, SPP1, TNFSF11, TNFRSF11B, PTHLH
48cysteine447.3CSF1R, TNFSF11, PDGFRB, TNFRSF11B, IBSP, BGLAP
49estrogen447.1IBSP, PDGFRB, CSF1, PTHLH, SPP1, BGLAP
50paraffin447.0ACP5, MMP1, MMP9, TNFSF11, IBSP, BGLAP

GO Terms for genes affiliated with Pigmented Villonodular Synovitis

About this section

Cellular components related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1membrane-bounded vesicleGO:03198810.0BGLAP, SPP1
2extracellular regionGO:0055768.4MMP1, SPP1, BGLAP, IBSP, TNFSF11, TNFRSF11B
3extracellular spaceGO:0056158.3SPP1, BGLAP, TNFSF11, TNFRSF11B, PTHLH, MMP9

Biological processes related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

(show all 18)
idNameGO IDScoreTop Affiliating Genes
1regulation of bone resorptionGO:04512410.2CSF1R, BGLAP
2osteoclast proliferationGO:00215810.1TNFSF11, CSF1
3positive regulation of bone resorptionGO:04578010.1TNFSF11, SPP1
4response to vitamin DGO:03328010.1BGLAP, SPP1
5phosphatidylinositol metabolic processGO:04648810.0PDGFRB, CSF1R
6biomineral tissue developmentGO:03121410.0IBSP, SPP1
7osteoblast developmentGO:0020769.9BGLAP, PTHLH
8osteoclast differentiationGO:0303169.9CSF1, CSF1R, TNFSF11
9positive regulation of osteoclast differentiationGO:0456729.9TNFSF11, CSF1
10extracellular matrix disassemblyGO:0226179.8SPP1, MMP9, MMP1
11macrophage differentiationGO:0302259.8MMP9, CSF1R, CSF1
12skeletal system developmentGO:0015019.7MMP9, PTHLH, TNFRSF11B, BGLAP
13bone resorptionGO:0454539.7ACP5, TNFSF11
14ossificationGO:0015039.7CSF1, MMP9, TNFSF11, IBSP
15positive regulation of cell migrationGO:0303359.6CSF1R, CSF1, PDGFRB
16extracellular matrix organizationGO:0301989.5SPP1, IBSP, TNFRSF11B, MMP9, MMP1
17phosphatidylinositol-mediated signalingGO:0480159.4PDGFRB, CSF1R, PCNA
18positive regulation of cell proliferationGO:0082849.3PTHLH, CSF1R, CSF1, PDGFRB

Molecular functions related to Pigmented Villonodular Synovitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051259.4CSF1, TNFRSF11B, TNFSF11, SPP1

Products for genes affiliated with Pigmented Villonodular Synovitis

About this section
  • Antibodies
  • Proteins
  • Lysates

Sources for Pigmented Villonodular Synovitis

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet