MCID: FBR019
MIFTS: 47

Fibromatosis malady

Rare diseases category
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Summaries for Fibromatosis

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NIH Rare Diseases:42 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:65 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:
42NIH Rare Diseases, 44Novoseek, 62UMLS
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Fibromatosis, Aliases & Descriptions:

Name: Fibromatosis 42 44 62


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/GeneDecks gene sharing:

(show top 50)    (show all 180)
idRelated DiseaseScoreTop Affiliating Genes
1infantile myofibromatosis31.1DES
2pseudosarcomatous fibromatosis30.9VIM, DES
3fibroma30.6DES, VIM
4gastrointestinal stromal tumor30.6VIM, DES
5periodontitis30.6CD36, MMP1
6spindle cell carcinoma30.4VIM, DES
7fibromatosis, gingival30.0MMP2, 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
18desmoid tumor10.8
19infantile digital fibromatosis10.8
20congenital generalized fibromatosis10.6
21gingival fibromatosis with hypertrichosis10.5
22osteofibrous dysplasia10.4CD36
23hypertrichosis10.4
24jones syndrome10.4
25adenomatoid tumor10.3VIM
26hyalinosis, inherited systemic10.3
27gingival fibromatosis, 210.3
28gingival fibromatosis, 310.3
29gingival fibromatosis, 410.3
30ledderhose disease10.3
31fasciitis10.3
32sarcoma10.3
33gingival fibromatosis with distinctive facies10.3
34gingival fibromatosis, 110.3
35laband syndrome10.3
36arthropathy10.2MMP1
37aggressive periodontitis10.2
38ramon syndrome10.2
39mental retardation10.2
40zimmermann-laband syndrome10.2
41localized scleroderma10.2MMP1, CD36
42epidermolysis bullosa dystrophica10.2MMP1, CD36
43hypertensive heart disease10.2MMP1, CD36
44osteogenesis imperfecta10.2CD36, SERPINH1
45cherubism10.2
46familial adenomatous polyposis10.2
47plantar fascial fibromatosis10.2
48peritonitis10.2
49fibromatosis multiple non ossifying10.2
50thoracic aortic aneurysm10.2MMP2, MMP1

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:

32
Breast, Skin, Bone, Colon, Tongue, Lymph node, Small intestine, Pancreas, Testes, Bone marrow, Brain, Spinal cord, Lung, Thyroid, Tonsil, Appendix

Animal Models for Fibromatosis or affiliated genes

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MGI Mouse Phenotypes related to Fibromatosis:

36
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 708)
idTitleAuthorsYear
1
Hereditary gingival fibromatosis and its management: 2-year follow-up. (25395783)
2014
2
Infantile digital fibromatosis. (23241193)
2013
3
Hereditary gingival fibromatosis: a review and a report of a rare case. (23533834)
2013
4
Nuclear GSK-3I^ segregation in desmoid-type fibromatosis. (23614534)
2013
5
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
6
Prognostic factors influencing event-free survival and treatments in desmoid-type fibromatosis: analysis from a large institution. (24119719)
2013
7
An important role for initial observation in the management of paediatric desmoid fibromatosis. (23098387)
2013
8
Plantar fibromatosis--topical review. (24043350)
2013
9
Hereditary nonsyndromic gingival fibromatosis: report of family case series. (24191204)
2013
10
Surgical treatment of a giant mesenteric fibromatosis mimicking a gastrointestinal stromal tumor: a case report and current treatment aspects. (23175930)
2012
11
Juvenile hyaline fibromatosis: a rare lesion. (22234127)
2011
12
Juvenile hyaline fibromatosis. (22345782)
2011
13
Fibromatosis of breast mimicking sarcoidosis. (21772596)
2011
14
Congenital infantile fibromatosis of the cheek: report of a rare case and differential diagnosis. (21658911)
2011
15
Aggressive fibromatosis in children: a changing approach. (21909066)
2011
16
Fibromatosis stem cells rather than bone-marrow mesenchymal stem cells recapitulate a murine model of fibromatosis. (21501590)
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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Expression patterns in normal tissues for genes affiliated with Fibromatosis

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/GeneDecks:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5MMP2, SOS1
2
Show member pathways
fatty acid beta-oxidation VI (peroxisome)37
9.4MMP1, CD36
39.3MMP2, MMP1
4
Show member pathways
9.3MMP2, MMP1
59.3MMP1, MMP2
69.3CD36, MMP2
7
Show member pathways
9.2DES, VIM
8
Show member pathways
Signaling by Aurora kinases37
Aurora C signaling37
9.2VIM, DES
99.0MMP2, SOS1, MMP1
10
Show member pathways
Signaling Pathways in Glioblastoma37
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/GeneDecks:

(show top 50)    (show all 95)
idCompoundScoreTop Affiliating Genes
1phxa 85449.7MMP1, MMP2
2halofuginone449.7CD36, MMP2
3cgs-27023a449.7MMP1, MMP2
4thps449.7MMP2, MMP1
5vgvapg449.7MMP1, MMP2
6prinomastat449.7MMP1, MMP2
7ilomastat449.6MMP1, MMP2
8orcein449.5DES, VIM
9s 100449.5VIM, DES
10cilomilast449.4MMP1, MMP2
1111-deoxy-pge1449.4MMP1, CD36
12latanoprost44 1110.4MMP2, MMP1
13bafilomycin a144 61 1111.4MMP2, SERPINH1, DES
14(4-aminophenyl)mercuric acetate449.3MMP1, MMP2, CD36
15zoledronic acid449.2MMP1, MMP2, CD36
16alendronate44 50 1111.2MMP2, CD36, MMP1
17batimastat44 61 1111.2MMP1, MMP2, CD36
18hydroxyproline44 24 1111.2MMP2, MMP1, CD36
19pioglitazone28 44 50 1112.2CD36, MMP2, MMP1
20dermatan sulfate449.1VIM, DES, CD36
21hematoxylin449.1VIM, MMP1, DES
22progestin449.0MMP1, VIM, DES
23rosiglitazone28 44 50 24 1113.0CD36, MMP2, MMP1
24troglitazone44 28 61 1112.0MMP2, MMP1, CD36
25silicone448.9MMP2, VIM, CD36
26adriamycin448.9CD36, DES, VIM
27azathioprine44 2 50 1111.9VIM, DES, MMP2
28ascorbic acid44 249.8MMP1, CD36, SERPINH1, MMP2
29herbimycin a44 619.5MMP2, MMP1, CD36
3012-o-tetradecanoylphorbol 13-acetate448.5MMP1, DES, MMP2, VIM
31proline448.5DES, VIM, SERPINH1, MMP2
32hydrocortisone44 2 61 1111.4MMP1, MMP2, CD36, VIM
33cytochalasin d44 619.4MMP1, CD36, VIM, MMP2
34tgf beta1448.4MMP2, MMP1, VIM, CD36
35matrigel448.4VIM, MMP1, CD36, MMP2
36sb 20358044 619.4CD36, VIM, MMP1, MMP2
37lactate448.4DES, VIM, MMP1, MMP2
38genistein44 28 61 2 24 1113.3CD36, VIM, MMP1, MMP2
39progesterone44 28 61 24 1112.1DES, MMP2, MMP1, VIM
40hyaluronic acid44 249.0VIM, DES, MMP2, CD36, MMP1
41paraffin448.0CD36, MMP1, MMP2, DES, VIM
42creatinine448.0CD36, MMP1, DES, VIM, MMP2
43h2o2448.0MMP2, CD36, DES, MMP1, VIM
44estrogen447.9DES, VIM, MMP2, MMP1, CD36
45retinoic acid44 248.9CD36, MMP1, MMP2, VIM, DES
46cysteine447.9CD36, VIM, DES, MMP2, MMP1
47cycloheximide447.9MMP2, MMP1, CD36, VIM
48procollagen447.6MMP1, DES, CD36, SERPINH1, VIM, MMP2
49vegf447.6VIM, DES, SERPINH1, MMP2, MMP1, CD36
50serine447.2CD36, MMP1, MMP2, SOS1, DES, VIM

GO Terms for genes affiliated with Fibromatosis

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Biological processes related to Fibromatosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1muscle filament slidingGO:0300499.1VIM, DES
2blood coagulationGO:0075969.1CD36, MMP1, SOS1
3extracellular matrix organizationGO:0301988.6MMP1, 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
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet