MCID: FBR019
MIFTS: 40

Fibromatosis malady

Rare diseases category

Summaries for Fibromatosis

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NIH Rare Diseases:41 Fibromatosis refers to a group of conditions characterized by overgrowths of skin and connective tissue called fibromas. these fibromas are usually benign (non-cancerous). fibromatosis can be classified by a person's age or by the location of the fibromas. the cause of fibromatosis is often unknown, and treatment depends on the individual disease. last updated: 5/10/2010

MalaCards based summary: Fibromatosis is related to infantile myofibromatosis and pseudosarcomatous fibromatosis. An important gene associated with Fibromatosis is SOS1 (son of sevenless homolog 1 (Drosophila)), and among its related pathways are Plasma membrane estrogen receptor signaling and PPAR signaling pathway. The compounds phxa 85 and halofuginone have been mentioned in the context of this disorder. Affiliated tissues include breast, skin and bone, and related mouse phenotypes are vision/eye and muscle.

Wikipedia:63 The term fibromatosis refers to a group of benign soft tissue tumors which have certain characteristics... more...

Aliases & Classifications for Fibromatosis

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Sources:
41NIH Rare Diseases, 43Novoseek, 60UMLS
See all sources

Fibromatosis, Aliases & Descriptions:

Name: Fibromatosis 41 43 60


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases


Related Diseases for Fibromatosis

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Diseases related to Fibromatosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 173)
idRelated DiseaseScoreTop Affiliating Genes
1infantile myofibromatosis31.1DES
2pseudosarcomatous fibromatosis30.9VIM, DES
3periodontitis30.6CD36, MMP1
4fibroma30.6DES, VIM
5gastrointestinal stromal tumor30.6VIM, DES
6spindle cell carcinoma30.5VIM, DES
7fibromatosis, gingival30.2MMP2, SOS1, MMP1, SERPINH1, CD36
8fibrous histiocytoma30.0DES, VIM
9arthritis29.9MMP2, MMP1
10gingival overgrowth29.9ANTXR2, MMP1, CD36
11keloids29.8MMP1, SERPINH1, CD36
12leiomyoma29.7VIM, DES
13rheumatoid arthritis29.4MMP2, SERPINH1, MMP1, CD36
14fibrosarcoma29.2MMP1, MMP2, DES, CD36, VIM
15melanoma28.9DES, VIM, MMP2, CD36, MMP1
16juvenile hyaline fibromatosis10.9
17gingivitis10.9
18infantile digital fibromatosis10.8
19hypertrichosis terminalis, generalized, with or without gingival hyperplasia10.6
20hyaline fibromatosis syndrome10.5
21desmoid disease, hereditary10.5
22hypertrichosis10.4
23fibrous dysplasia10.4CD36
24jones syndrome10.4
25fibromatosis, gingival, 310.4
26zimmermann-laband syndrome10.4
27fibromatosis, gingival, 210.4
28fibromatosis, gingival, 410.4
29gingival fibromatosis with distinctive facies10.3
30adenomatoid tumor10.3VIM
31sarcoma10.3
32ledderhose disease10.3
33ramon syndrome10.3
34aggressive periodontitis10.3
35fasciitis10.3
36arthropathy10.2MMP1
37fibromatosis multiple non ossifying10.2
38mental retardation10.2
39localized scleroderma10.2MMP1, CD36
40epidermolysis bullosa dystrophica10.2MMP1, CD36
41chronic ulcer of skin10.2MMP1, CD36
42hypertensive heart disease10.2MMP1, CD36
43cherubism10.2
44plantar fascial fibromatosis10.2
45peritonitis10.2
46osteogenesis imperfecta10.2CD36, SERPINH1
47thoracic aortic aneurysm10.2MMP2, MMP1
48conjunctivochalasis10.1MMP2, MMP1
49epithelioid leiomyosarcoma10.1DES, VIM
50mesenchymal chondrosarcoma10.1DES, VIM

Graphical network of the top 20 diseases related to Fibromatosis:



Diseases related to fibromatosis

Symptoms for Fibromatosis

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Drugs & Therapeutics for Fibromatosis

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Drug clinical trials:

Search ClinicalTrials for Fibromatosis

Search NIH Clinical Center for Fibromatosis

Genetic Tests for Fibromatosis

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Anatomical Context for Fibromatosis

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MalaCards organs/tissues related to Fibromatosis:

31
Breast, Skin, Bone, Colon, Tongue, Lymph node, Small intestine, Pancreas, Testes, Brain, Spinal cord, Lung, Thyroid, Tonsil, Appendix

Animal Models for Fibromatosis or affiliated genes

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MGI Mouse Phenotypes related to Fibromatosis:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053918.2CD36, MMP2, VIM, SOS1
2MP:00053697.8DES, SOS1, VIM, MMP2, CD36
3MP:00053877.2SOS1, VIM, MMP2, MMP1, ANTXR2, CD36
4MP:00053857.1CD36, DES, SERPINH1, SOS1, VIM, MMP2
5MP:00107687.0DES, SERPINH1, SOS1, VIM, MMP2, ANTXR2
6MP:00053767.0DES, SOS1, VIM, MMP2, MMP1, CD36

Publications for Fibromatosis

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Articles related to Fibromatosis:

(show top 50)    (show all 725)
idTitleAuthorsYear
1
Prevalence of palmar fibromatosis with and without contracture in asymptomatic patients. (25854281)
2015
2
Hereditary gingival fibromatosis and its management: 2-year follow-up. (25395783)
2014
3
Infantile digital fibromatosis. (23241193)
2013
4
Hereditary gingival fibromatosis: a review and a report of a rare case. (23533834)
2013
5
Nuclear GSK-3I^ segregation in desmoid-type fibromatosis. (23614534)
2013
6
Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm--a position paper from the Italian and the French Sarcoma Group. (24325833)
2013
7
Prognostic factors influencing event-free survival and treatments in desmoid-type fibromatosis: analysis from a large institution. (24119719)
2013
8
An important role for initial observation in the management of paediatric desmoid fibromatosis. (23098387)
2013
9
Plantar fibromatosis--topical review. (24043350)
2013
10
Hereditary nonsyndromic gingival fibromatosis: report of family case series. (24191204)
2013
11
Surgical treatment of a giant mesenteric fibromatosis mimicking a gastrointestinal stromal tumor: a case report and current treatment aspects. (23175930)
2012
12
Juvenile hyaline fibromatosis: a rare lesion. (22234127)
2011
13
Juvenile hyaline fibromatosis. (22345782)
2011
14
Fibromatosis of breast mimicking sarcoidosis. (21772596)
2011
15
Congenital infantile fibromatosis of the cheek: report of a rare case and differential diagnosis. (21658911)
2011
16
Aggressive fibromatosis in children: a changing approach. (21909066)
2011
17
Limb salvage surgery in the excision of a massive fibromatosis. (21871256)
2011
18
Objective response to hydroxyurea in a patient with heavily pre-treated aggressive fibromatosis. (20658640)
2010
19
Spectrum of mutations in the ANTXR2 (CMG2) gene in infantile systemic hyalinosis and juvenile hyaline fibromatosis. (20331448)
2010
20
Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). (20187095)
2010
21
The presentation and management of fibromatosis colli. (20859860)
2010
22
Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only. (21075698)
2010
23
Aggressive fibromatosis of the chest wall: sonographic appearance of the fascial tail and staghorn patterns. (19244079)
2009
24
Infantile digital fibroma/fibromatosis: a clinicopathologic and immunohistochemical study of 69 tumors from 57 patients with long-term follow-up. (18830128)
2009
25
Idiopathic gingival fibromatosis: case report and its management. (20339448)
2009
26
Clinical and histomorphometric characteristics of three different families with hereditary gingival fibromatosis. (18061489)
2008
27
Fibromatosis of the breast after mammary prosthesis implantation. (18827791)
2008
28
Natural course of desmoid-type fibromatosis. (18274856)
2008
29
The fibromatosis signature defines a robust stromal response in breast carcinoma. (18414401)
2008
30
Congenital desmoid fibromatosis in a Holstein heifer. (19043487)
2008
31
Ovarian fibromatosis: magnetic resonance imaging findings with pathologic correlation. (18830110)
2008
32
Desmoid-type fibromatosis involving the brachial plexus. (17613214)
2007
33
Aggressive fibromatosis: MRI features with pathologic correlation. (16357411)
2006
34
Plasminogen activator inhibitor-1 (PAI-1) modifies the formation of aggressive fibromatosis (desmoid tumor). (15674349)
2005
35
Gingival fibromatosis, short stature, border-line IQ, facial dysmorphism and hepatomegaly. (16080296)
2005
36
Juvenile hyaline fibromatosis. (15235211)
2004
37
Role of the c-myc proto-oncogene in the proliferation of hereditary gingival fibromatosis fibroblasts. (15088873)
2004
38
Genetic deletion of receptor for hyaluronan-mediated motility (Rhamm) attenuates the formation of aggressive fibromatosis (desmoid tumor). (14534534)
2003
39
Low-grade (fibromatosis-like) spindle cell carcinoma of the breast. (11474284)
2001
40
Predominance of beta-catenin mutations and beta-catenin dysregulation in sporadic aggressive fibromatosis (desmoid tumor). (10597266)
1999
41
Congenital generalized fibromatosis. Extraosseous accumulation of bone seeking radiopharmaceutical. (8732847)
1996
42
TGF beta and bFGF synthesis and localization in Dupuytren's disease (nodular palmar fibromatosis) relative to cellular activity, myofibroblast phenotype and oncofetal variants of fibronectin. (8789403)
1995
43
Hypertrichosis universalis congenita: a separate entity, or the same disease as gingival fibromatosis? (8415305)
1993
44
Extra-abdominal fibromatosis. An immunohistochemical analysis]. (8113044)
1993
45
Juvenile hyaline fibromatosis. (3686711)
1987
46
Congenital generalized fibromatosis: a new management strategy provided by magnetic resonance imaging. (3591755)
1987
47
Juvenile hyaline fibromatosis. (2433666)
1987
48
Infantile digital fibromatosis. Ultrastructural, histochemical, and tissue culture observations. (6253042)
1980
49
Congenital generalized fibromatosis: a case report with roentgen manifestations of the skeleton. (819961)
1976
50
Infantile digital fibromatosis. (4431801)
1974

Variations for Fibromatosis

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Expression for genes affiliated with Fibromatosis

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Search GEO for disease gene expression data for Fibromatosis.

Pathways for genes affiliated with Fibromatosis

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Pathways related to Fibromatosis according to GeneCards Suite gene sharing:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5MMP2, SOS1
2
Show member pathways
fatty acid beta-oxidation VI (peroxisome)36
9.4CD36, MMP1
39.3MMP1, MMP2
4
Show member pathways
9.3MMP1, MMP2
59.3MMP1, MMP2
69.3CD36, MMP2
7
Show member pathways
9.2VIM, DES
8
Show member pathways
Signaling by Aurora kinases36
Aurora C signaling36
9.2VIM, DES
99.0MMP1, MMP2, SOS1
10
Show member pathways
Signaling Pathways in Glioblastoma36
9.0SOS1, MMP2, MMP1
119.0SOS1, MMP2, MMP1
12
Show member pathways
8.9SERPINH1, MMP2, MMP1
13
Show member pathways
8.5CD36, MMP1, MMP2, SOS1

Compounds for genes affiliated with Fibromatosis

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Compounds related to Fibromatosis according to GeneCards Suite gene sharing:

(show top 50)    (show all 95)
idCompoundScoreTop Affiliating Genes
1phxa 85439.7MMP1, MMP2
2halofuginone439.7CD36, MMP2
3cgs-27023a439.7MMP1, MMP2
4thps439.7MMP2, MMP1
5vgvapg439.7MMP1, MMP2
6prinomastat439.7MMP1, MMP2
7ilomastat439.6MMP1, MMP2
8orcein439.5DES, VIM
9s 100439.5VIM, DES
10cilomilast439.4MMP1, MMP2
1111-deoxy-pge1439.4MMP1, CD36
12latanoprost43 1210.4MMP2, MMP1
13bafilomycin a143 59 1211.4MMP2, SERPINH1, DES
14(4-aminophenyl)mercuric acetate439.3MMP1, MMP2, CD36
15zoledronic acid439.2MMP1, MMP2, CD36
16alendronate43 49 1211.2MMP2, CD36, MMP1
17batimastat43 59 1211.2MMP1, MMP2, CD36
18hydroxyproline43 24 1211.2MMP2, MMP1, CD36
19pioglitazone28 43 49 1212.2CD36, MMP2, MMP1
20dermatan sulfate439.1VIM, DES, CD36
21hematoxylin439.1VIM, MMP1, DES
22progestin439.0MMP1, VIM, DES
23rosiglitazone28 43 49 24 1213.0CD36, MMP2, MMP1
24troglitazone43 28 59 1212.0MMP2, MMP1, CD36
25silicone438.9MMP2, VIM, CD36
26adriamycin438.9CD36, DES, VIM
27azathioprine43 2 49 1211.9VIM, DES, MMP2
28ascorbic acid43 249.8MMP1, CD36, SERPINH1, MMP2
29herbimycin a43 599.5MMP2, MMP1, CD36
3012-o-tetradecanoylphorbol 13-acetate438.5MMP1, DES, MMP2, VIM
31proline438.5DES, VIM, SERPINH1, MMP2
32hydrocortisone43 2 59 1211.4MMP1, MMP2, CD36, VIM
33cytochalasin d43 599.4MMP1, CD36, VIM, MMP2
34tgf beta1438.4MMP2, MMP1, VIM, CD36
35matrigel438.4VIM, MMP1, CD36, MMP2
36sb 20358043 599.4CD36, VIM, MMP1, MMP2
37lactate438.4DES, VIM, MMP1, MMP2
38genistein43 28 59 2 24 1213.3CD36, VIM, MMP1, MMP2
39progesterone43 28 59 24 1212.1DES, MMP2, MMP1, VIM
40hyaluronic acid43 249.0VIM, DES, MMP2, CD36, MMP1
41paraffin438.0CD36, MMP1, MMP2, DES, VIM
42creatinine438.0CD36, MMP1, DES, VIM, MMP2
43h2o2438.0MMP2, CD36, DES, MMP1, VIM
44estrogen437.9DES, VIM, MMP2, MMP1, CD36
45retinoic acid43 248.9CD36, MMP1, MMP2, VIM, DES
46cysteine437.9CD36, VIM, DES, MMP2, MMP1
47cycloheximide437.9MMP2, MMP1, CD36, VIM
48procollagen437.6MMP1, DES, CD36, SERPINH1, VIM, MMP2
49vegf437.6VIM, DES, SERPINH1, MMP2, MMP1, CD36
50serine437.2CD36, MMP1, MMP2, SOS1, DES, VIM

GO Terms for genes affiliated with Fibromatosis

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Biological processes related to Fibromatosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1muscle filament slidingGO:00300499.1VIM, DES
2blood coagulationGO:00075969.1CD36, MMP1, SOS1
3extracellular matrix organizationGO:00301988.6MMP1, MMP2, SERPINH1

Products for genes affiliated with Fibromatosis

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Sources for Fibromatosis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet