MCID: FBR019
MIFTS: 47

Fibromatosis malady

Rare diseases category
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Summaries for Fibromatosis

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43NIH Rare Diseases, 65Wikipedia, 33MalaCards
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NIH Rare Diseases:43 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: Fibromatosis is related to pseudosarcomatous fibromatosis and gastrointestinal stromal tumor. 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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43NIH Rare Diseases, 45Novoseek, 62UMLS
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Classifications:

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


Aliases & Descriptions:

fibromatosis 43 45 62


Related Diseases for Fibromatosis

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17GeneCards, 18GeneDecks
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Diseases related to Fibromatosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 176)
idRelated DiseaseScoreTop Affiliating Genes
1pseudosarcomatous fibromatosis30.7VIM, DES
2gastrointestinal stromal tumor30.5VIM, DES
3infantile myofibromatosis30.5DES
4fibroma30.5DES, VIM
5periodontitis30.5CD36, MMP1
6fibromatosis, gingival30.4MMP2, SOS1, MMP1, SERPINH1, CD36
7spindle cell carcinoma30.3VIM, DES
8fibrous histiocytoma29.8DES, VIM
9arthritis29.8MMP2, MMP1
10gingival overgrowth29.8ANTXR2, MMP1, CD36
11fibrosarcoma29.8MMP1, MMP2, DES, CD36, VIM
12keloids29.8MMP1, SERPINH1, CD36
13leiomyoma29.8VIM, DES
14rheumatoid arthritis29.7MMP2, SERPINH1, MMP1, CD36
15melanoma29.6DES, VIM, MMP2, CD36, MMP1
16juvenile hyaline fibromatosis10.9
17gingivitis10.9
18infantile digital fibromatosis10.8
19desmoid tumor10.7
20congenital generalized fibromatosis10.6
21gingival fibromatosis with hypertrichosis10.5
22hypertrichosis10.4
23plantar fascial fibromatosis10.3
24gingival fibromatosis, 210.3
25gingival fibromatosis, 310.3
26gingival fibromatosis, 410.3
27fasciitis10.3
28hyalinosis, inherited systemic10.3
29gingival fibromatosis with distinctive facies10.3
30gingival fibromatosis, 110.3
31jones syndrome10.3
32laband syndrome10.3
33aggressive periodontitis10.2
34sarcoma10.2
35ramon syndrome10.2
36mental retardation10.2
37zimmermann-laband syndrome10.2
38cherubism10.2
39familial adenomatous polyposis10.2
40peritonitis10.2
41fibromatosis multiple non ossifying10.2
42amelogenesis imperfecta and gingival fibromatosis syndrome10.1
43phyllode tumor10.1
44gingival fibromatosis - facial dysmorphism10.1
45familial dupuytren contracture10.1
46osteofibrous dysplasia10.1CD36
47adenomatoid tumor10.1VIM
48arthropathy10.1MMP1
49localized scleroderma10.0MMP1, CD36
50epidermolysis bullosa dystrophica10.0MMP1, CD36

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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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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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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33MalaCards
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MalaCards organs/tissues related to Fibromatosis:

33
Breast, Skin, Bone, Colon, Tongue, Pancreas, Lymph node, Small intestine, Tonsil, Thyroid, Bone marrow, Testes, Lung, Spinal cord, Brain, Appendix

Animal Models for Fibromatosis or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Fibromatosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053918.2CD36, SOS1, VIM, MMP2
2MP:00053697.8SOS1, VIM, MMP2, DES, CD36
3MP:00053877.2ANTXR2, CD36, MMP1, MMP2, VIM, SOS1
4MP:00053857.1DES, SERPINH1, SOS1, VIM, MMP2, ANTXR2
5MP:00107687.0CD36, MMP2, VIM, SOS1, SERPINH1, DES
6MP:00053767.0DES, CD36, MMP1, MMP2, VIM, SOS1

Publications for Fibromatosis

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52PubMed
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Articles related to Fibromatosis:

(show top 50)    (show all 682)
idTitleAuthorsYear
1
Infantile digital fibromatosis. (23241193)
2013
2
Hereditary gingival fibromatosis: a review and a report of a rare case. (23533834)
2013
3
Nuclear GSK-3I^ segregation in desmoid-type fibromatosis. (23614534)
2013
4
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
5
Prognostic factors influencing event-free survival and treatments in desmoid-type fibromatosis: analysis from a large institution. (24119719)
2013
6
An important role for initial observation in the management of paediatric desmoid fibromatosis. (23098387)
2013
7
Plantar fibromatosis--topical review. (24043350)
2013
8
Hereditary nonsyndromic gingival fibromatosis: report of family case series. (24191204)
2013
9
Desmoid-type fibromatosis in the head and neck: CT and MR imaging characteristics. (23338838)
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
Abnormalities of chromosome 8, APC and beta-catenin genes in aggressive fibromatosis]. (18646694)
2008
33
Desmoid-type fibromatosis involving the brachial plexus. (17613214)
2007
34
Aggressive fibromatosis: MRI features with pathologic correlation. (16357411)
2006
35
Plasminogen activator inhibitor-1 (PAI-1) modifies the formation of aggressive fibromatosis (desmoid tumor). (15674349)
2005
36
Gingival fibromatosis, short stature, border-line IQ, facial dysmorphism and hepatomegaly. (16080296)
2005
37
Juvenile hyaline fibromatosis. (15235211)
2004
38
Role of the c-myc proto-oncogene in the proliferation of hereditary gingival fibromatosis fibroblasts. (15088873)
2004
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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2BioGPS, 15Gene Expression Omnibus DataSets
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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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50PathCards, 38NCBI BioSystems Database, 30KEGG, 55Reactome, 53QIAGEN, 54R&D Systems
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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)38
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 kinases38
Aurora C signaling38
9.2VIM, DES
99.0MMP2, SOS1, MMP1
10
Show member pathways
Signaling Pathways in Glioblastoma38
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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45Novoseek, 11DrugBank, 61Tocris Bioscience, 51PharmGKB, 24HMDB, 29IUPHAR, 3BitterDB
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Compounds related to Fibromatosis according to GeneCards/GeneDecks:

(show top 50)    (show all 95)
idCompoundScoreTop Affiliating Genes
1phxa 85459.7MMP1, MMP2
2halofuginone459.7CD36, MMP2
3cgs-27023a459.7MMP1, MMP2
4thps459.7MMP2, MMP1
5vgvapg459.7MMP1, MMP2
6prinomastat459.7MMP1, MMP2
7ilomastat459.6MMP1, MMP2
8orcein459.5DES, VIM
9s 100459.5VIM, DES
10cilomilast459.4MMP1, MMP2
1111-deoxy-pge1459.4MMP1, CD36
12latanoprost45 1110.4MMP2, MMP1
13bafilomycin a145 61 1111.4MMP2, SERPINH1, DES
14(4-aminophenyl)mercuric acetate459.3MMP1, MMP2, CD36
15zoledronic acid459.2MMP1, MMP2, CD36
16alendronate45 51 1111.2MMP2, CD36, MMP1
17batimastat45 61 1111.2MMP1, MMP2, CD36
18hydroxyproline45 24 1111.2MMP2, MMP1, CD36
19pioglitazone29 45 51 1112.2CD36, MMP2, MMP1
20dermatan sulfate459.1VIM, DES, CD36
21hematoxylin459.1VIM, MMP1, DES
22progestin459.0MMP1, VIM, DES
23rosiglitazone29 45 51 24 1113.0CD36, MMP2, MMP1
24troglitazone45 29 61 1112.0MMP2, MMP1, CD36
25silicone458.9MMP2, VIM, CD36
26adriamycin458.9CD36, DES, VIM
27azathioprine45 3 51 1111.9VIM, DES, MMP2
28ascorbic acid45 249.8MMP1, CD36, SERPINH1, MMP2
29herbimycin a45 619.5MMP2, MMP1, CD36
3012-o-tetradecanoylphorbol 13-acetate458.5MMP1, DES, MMP2, VIM
31proline458.5DES, VIM, SERPINH1, MMP2
32hydrocortisone45 3 61 1111.4MMP1, MMP2, CD36, VIM
33cytochalasin d45 619.4MMP1, CD36, VIM, MMP2
34tgf beta1458.4MMP2, MMP1, VIM, CD36
35matrigel458.4VIM, MMP1, CD36, MMP2
36sb 20358045 619.4CD36, VIM, MMP1, MMP2
37lactate458.4DES, VIM, MMP1, MMP2
38genistein45 29 61 3 24 1113.3CD36, VIM, MMP1, MMP2
39progesterone45 29 61 24 1112.1DES, MMP2, MMP1, VIM
40hyaluronic acid45 249.0VIM, DES, MMP2, CD36, MMP1
41paraffin458.0CD36, MMP1, MMP2, DES, VIM
42creatinine458.0CD36, MMP1, DES, VIM, MMP2
43h2o2458.0MMP2, CD36, DES, MMP1, VIM
44estrogen457.9DES, VIM, MMP2, MMP1, CD36
45retinoic acid45 248.9CD36, MMP1, MMP2, VIM, DES
46cysteine457.9CD36, VIM, DES, MMP2, MMP1
47cycloheximide457.9MMP2, MMP1, CD36, VIM
48procollagen457.6MMP1, DES, CD36, SERPINH1, VIM, MMP2
49vegf457.6VIM, DES, SERPINH1, MMP2, MMP1, CD36
50serine457.2CD36, MMP1, MMP2, SOS1, DES, VIM

GO Terms for genes affiliated with Fibromatosis

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16Gene Ontology
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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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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet