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MCID: FBR003
MIFTS: 43

Fibrous Histiocytoma

Categories: Bone diseases, Cancer diseases, Immune diseases, Rare diseases, Skin diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Fibrous Histiocytoma

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MalaCards integrated aliases for Fibrous Histiocytoma:

Name: Fibrous Histiocytoma 12 15
Histiocytoma, Benign Fibrous 44 70
Benign Fibrous Histiocytoma 12
Histiocytoma Fibrous 54
Fibroxanthoma 12

Classifications:

MalaCards categories:
Global: Cancer diseases Rare diseases
Anatomical: Skin diseases Bone diseases Immune diseases
See all MalaCards categories (disease lists)


External Ids:

Disease Ontology 12 DOID:4415
MeSH 44 D018219
NCIt 50 C3739
SNOMED-CT 67 25889007
UMLS 70 C0206644
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Summaries for Fibrous Histiocytoma

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MalaCards based summary : Fibrous Histiocytoma, also known as histiocytoma, benign fibrous, is related to malignant fibrous histiocytoma of bone and malignant inflammatory fibrous histiocytoma. An important gene associated with Fibrous Histiocytoma is ATF1 (Activating Transcription Factor 1), and among its related pathways/superpathways are Transcriptional misregulation in cancer and Striated Muscle Contraction. The drugs Etoposide and Vincristine have been mentioned in the context of this disorder. Affiliated tissues include bone, skin and pancreas, and related phenotypes are cellular and respiratory system

Wikipedia : 73 A dermatofibroma, or benign fibrous histiocytomas, is a benign skin... more...

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Related Diseases for Fibrous Histiocytoma

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Diseases in the Fibrous Histiocytoma family:

Malignant Fibrous Histiocytoma Malignant Inflammatory Fibrous Histiocytoma

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

(show top 50) (show all 710)
# Related Disease Score Top Affiliating Genes
1 malignant fibrous histiocytoma of bone 32.8 SERPINA3 DES
2 malignant inflammatory fibrous histiocytoma 32.4 SERPINA3 CD68
3 malignant fibrous histiocytoma 31.9 VIM SERPINA3 S100B MTAP MFHAS1 F13A1
4 tenosynovial giant cell tumor 31.8 SERPINA3 CD68 CD34 CD163
5 rare tumor 31.6 EWSR1 DES ACTC1
6 cutaneous fibrous histiocytoma 31.5 VIM SERPINA3 S100B F13A1 DES CD68
7 myxofibrosarcoma 31.3 SERPINA3 S100B MTAP FUS EWSR1 DES
8 myxoid liposarcoma 31.0 FUS EWSR1 ATF1
9 fibrosarcoma 30.9 VIM FUS EWSR1 DES ALK ACTC1
10 malignant mesenchymoma 30.8 SERPINA3 DES CD34
11 bone sarcoma 30.8 SERPINA3 MTAP EWSR1
12 embryonal sarcoma 30.7 VIM SERPINA3 S100B DES
13 liposarcoma 30.7 VIM FUS EWSR1 DES CD68 CD34
14 fibromatosis 30.6 VIM DES ACTC1
15 myoepithelioma 30.6 VIM S100B EWSR1 ACTC1
16 extraosseous chondrosarcoma 30.6 FUS EWSR1 ENO2
17 spindle cell sarcoma 30.5 VIM SERPINA3 S100B EWSR1 DES CD34
18 juvenile xanthogranuloma 30.5 SERPINA3 S100B F13A1
19 sarcoma 30.5 VIM S100B MTAP FUS EWSR1 ATF1
20 chordoma 30.5 VIM SERPINA3 S100B MTAP DES
21 well-differentiated liposarcoma 30.5 SERPINA3 FUS EWSR1 CD34
22 carcinosarcoma 30.5 VIM DES ACTC1
23 vulvar sarcoma 30.5 EWSR1 CD34
24 sarcomatosis 30.5 EWSR1 ATF1
25 lung sarcoma 30.5 SERPINA3 EWSR1 CD34
26 reticulum cell sarcoma 30.4 VIM DES ACTC1
27 xanthogranulomatous pyelonephritis 30.4 SERPINA3 CD68
28 histiocytic sarcoma 30.4 CD68 CD163
29 leiomyoma 30.4 VIM DES CD34 ACTC1
30 meningioma, familial 30.4 VIM SERPINA3 S100B CD68 CD34
31 malignant giant cell tumor 30.4 S100B CD68 ACTC1
32 castleman disease 30.4 S100B F13A1 DES
33 granuloma annulare 30.3 VIM F13A1 CD68
34 cystadenocarcinoma 30.3 VIM SERPINA3 ACTC1
35 extraosseous osteosarcoma 30.3 CD68 CD34
36 pleomorphic lipoma 30.3 SERPINA3 CD68 CD34
37 benign breast phyllodes tumor 30.3 VIM CD34
38 sclerosing liposarcoma 30.3 FUS CD34
39 leiomyosarcoma 30.3 VIM SERPINA3 S100B PECAM1 ENO2 DES
40 angiosarcoma 30.3 VIM PECAM1 CD68 CD34
41 extraskeletal chondroma 30.2 SERPINA3 EWSR1
42 breast sarcoma 30.2 PECAM1 CD34
43 fasciitis 30.2 VIM MITF CD34 ACTC1
44 rhabdoid cancer 30.2 VIM SERPINA3 EWSR1 DES ACTC1
45 meningothelial meningioma 30.2 SERPINA3 ENO2
46 benign giant cell tumor 30.2 VIM SERPINA3 MITF DES ACTC1
47 ectomesenchymoma 30.2 ENO2 DES
48 myxoid chondrosarcoma 30.2 S100B MTAP EWSR1 CD68
49 benign mesothelioma 30.2 VIM PECAM1 MTAP
50 chondrosarcoma 30.2 VIM SERPINA3 S100B MTAP EWSR1 ENO2

Graphical network of the top 20 diseases related to Fibrous Histiocytoma:



Diseases related to Fibrous Histiocytoma
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Symptoms & Phenotypes for Fibrous Histiocytoma

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MGI Mouse Phenotypes related to Fibrous Histiocytoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.7 ACTC1 ATF1 CD163 CD34 CREB1 CREM
2 respiratory system MP:0005388 9.28 ALK CD163 CREB1 CREM ENO2 F13A1
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Drugs & Therapeutics for Fibrous Histiocytoma

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Drugs for Fibrous Histiocytoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 56)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Etoposide Approved Phase 3 33419-42-0 36462
2
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
3
Epirubicin Approved Phase 3 56420-45-2 41867
4
Dactinomycin Approved, Investigational Phase 3 50-76-0 2019 457193
5
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
6
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
7
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
8
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
9
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
10
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
11 Immunosuppressive Agents Phase 3
12 Tubulin Modulators Phase 3
13 Anti-Infective Agents Phase 3
14 Antirheumatic Agents Phase 3
15 Etoposide phosphate Phase 3
16 Antimitotic Agents Phase 3
17 Antibiotics, Antitubercular Phase 2, Phase 3
18 Alkylating Agents Phase 2, Phase 3
19 Anti-Bacterial Agents Phase 2, Phase 3
20
Isophosphamide mustard Phase 2, Phase 3 100427
21
Liposomal doxorubicin Phase 2, Phase 3 31703
22
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
23
Bevacizumab Approved, Investigational Phase 2 216974-75-3
24
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
25
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
26
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
27
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
28
Levoleucovorin Approved, Investigational Phase 2 68538-85-2 149436
29
Tyrosine Approved, Investigational, Nutraceutical Phase 2 60-18-4 6057
30
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
31
Saracatinib Investigational Phase 2 379231-04-6
32 Imatinib Mesylate Phase 2 220127-57-1 123596
33 Protein Kinase Inhibitors Phase 2
34 Angiogenesis Inhibitors Phase 2
35 Immunologic Factors Phase 2
36 Antineoplastic Agents, Immunological Phase 2
37 Immunoglobulins Phase 2
38 Folic Acid Antagonists Phase 2
39 Mitogens Phase 2
40 Vitamin B9 Phase 2
41 Antibodies Phase 2
42 Vitamin B Complex Phase 2
43 Antibodies, Monoclonal Phase 2
44 Antimetabolites Phase 2
45 Folate Phase 2
46 Dermatologic Agents Phase 2
47 Endothelial Growth Factors Phase 2
48
Celecoxib Approved, Investigational Phase 1 169590-42-5 2662
49
nivolumab Approved Phase 1 946414-94-4
50 Analgesics, Non-Narcotic Phase 1

Interventional clinical trials:

(show all 21)
# Name Status NCT ID Phase Drugs
1 Localized Non-Rhabdomyosarcoma Soft Tissue Sarcomas Unknown status NCT00334854 Phase 3 doxorubicin hydrochloride;ifosfamide
2 Risk-Based Treatment for Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) in Patients Under 30 Years of Age Unknown status NCT00346164 Phase 3 doxorubicin hydrochloride;ifosfamide
3 MMT 95 Study For Rhabdomyosarcoma and Other Malignant Soft Tissue Tumors of Childhood Completed NCT00002898 Phase 3 carboplatin;cyclophosphamide;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
4 Pazopanib Neoadjuvant Trial in Non-Rhabdomyosarcoma Soft Tissue Sarcomas (PAZNTIS): A Phase II/III Randomized Trial of Preoperative Chemoradiation or Preoperative Radiation Plus or Minus Pazopanib (NSC# 737754) Active, not recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin;Doxorubicin Hydrochloride;Ifosfamide;Pazopanib;Pazopanib Hydrochloride
5 Phase II Trial of Gleevec (Formerly Known as STI571) in Patients With Soft Tissue and Bone Sarcomas: A Multi-Disciplinary Trial of the North American Sarcoma Study Group of the Connective Tissue Oncology Society Completed NCT00031915 Phase 2 imatinib mesylate
6 A Multicenter Phase II Study of Continuous Dosing of Sunitinib (Sutent®, SU11248) in Non-GIST Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
7 Phase II Open-Label Study of Sunitinib Malate (SU011248) in Adult Patients With Metastatic and/or Surgically Unresectable Soft Tissue Sarcoma Completed NCT00400569 Phase 2 Sunitinib Malate (SU011248)
8 A Phase II Study of Neoadjuvant Bevacizumab and Radiation Therapy for Resectable Soft Tissue Sarcomas Completed NCT00356031 Phase 2 Bevacizumab
9 A Multicenter Phase II Study of Sorafenib (BAY43-9006) in Non-GIST Sarcomas Completed NCT00245102 Phase 2 sorafenib tosylate
10 A Phase II Study of Single Agent Depsipeptide (FK228) in Metastatic or Unresectable Soft Tissue Sarcomas Completed NCT00112463 Phase 2 romidepsin
11 A Phase 2 Study of Intravenous REOLYSIN® (Wild-Type Reovirus) in the Treatment of Patients With Bone and Soft Tissue Sarcomas Metastatic to the Lung Completed NCT00503295 Phase 2
12 A Phase 2 Study of AZD0530 in Recurrent or Metastatic Soft Tissue Sarcoma Completed NCT00659360 Phase 2 saracatinib
13 A Study of Bevacizumab, a Humanized Monoclonal Antibody Against Vascular Endothelial Growth Factor (VEGF), in Combination With Chemotherapy for Treatment of Osteosarcoma Completed NCT00667342 Phase 2 Cisplatin;Doxorubicin;Methotrexate;Ifosfamide;etoposide
14 A Phase I Trial of Postoperative Radiation With Dose-Escalation of A Cox-2 Inhibitor, Celebrexâ„¢ (CELECOXIB) in Patients With Soft Tissue Sarcoma of the Extremity Completed NCT00450736 Phase 1 celecoxib
15 A Phase I Study of Nivolumab and Intratumoral BO 112 for Resectable Soft Tissue Sarcoma Recruiting NCT04420975 Phase 1 Immunotherapeutic Agent
16 Angiomatoid Fibrous Histiocytoma: a Single Institution Case-series Completed NCT03759327
17 Genetic Study of Patients and Families With Diaphyseal Medullary Stenosis With Malignant Fibrous Histiocytoma of the Bone Completed NCT00007046
18 A Pilot Study on the Effects of Preoperative Physical Therapy in Adolescents and Young Adults Diagnosed With a Lower Extremity Malignancy Completed NCT01674101
19 Optical Biopsy of Human Skin in Conjunction With Laser Treatment Completed NCT00540566
20 Efficacy of 1540 Nanometer Erbium Glass Laser to Improve Benign Dermatofibromas Active, not recruiting NCT03392935
21 Prevention of Mucositis in Children With AES-14 (IND#36978), a Glutamine Based Oral Care Regimen, for Patients Diagnosed With Solid Tumors: A Randomized Placebo-Controlled Clinical Study Withdrawn NCT00334984 glutamine

Search NIH Clinical Center for Fibrous Histiocytoma

Cochrane evidence based reviews: histiocytoma, benign fibrous

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Genetic Tests for Fibrous Histiocytoma

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Anatomical Context for Fibrous Histiocytoma

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MalaCards organs/tissues related to Fibrous Histiocytoma:

40
Bone, Skin, Pancreas, Colon, Spleen, Endothelial, Prostate
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Publications for Fibrous Histiocytoma

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Articles related to Fibrous Histiocytoma:

(show top 50) (show all 4626)
# Title Authors PMID Year
1
Primary intracerebral angiomatoid fibrous histiocytoma: report of a case with a t(12;22)(q13;q12) causing type 1 fusion of the EWS and ATF-1 genes. 54 61
18300800 2008
2
High proliferative activity excludes dermatofibroma: report of the utility of MIB-1 in the differential diagnosis of selected fibrohistiocytic tumors. 54 61
16740036 2006
3
Expression of CD163 in dermatofibroma, cellular fibrous histiocytoma, and dermatofibrosarcoma protuberans: comparison with CD68, CD34, and Factor XIIIa. 61 54
16640542 2006
4
Fusion of the EWSR1 and ATF1 genes without expression of the MITF-M transcript in angiomatoid fibrous histiocytoma. 54 61
15884099 2005
5
Fusion of the FUS and BBF2H7 genes in low grade fibromyxoid sarcoma. 61 54
12915480 2003
6
S100A6 expression in fibrohistiocytic lesions. 61 54
11401666 2001
7
Genetic characterization of angiomatoid fibrous histiocytoma identifies fusion of the FUS and ATF-1 genes induced by a chromosomal translocation involving bands 12q13 and 16p11. 61 54
11063792 2000
8
Fibrous histiocytoma and fibrous tissue tumors of the orbit. 54 61
10026737 1999
9
Verocay body--prominent cutaneous schwannoma. 61 54
9185909 1997
10
Expression of the human progenitor cell antigen CD34 (HPCA-1) distinguishes dermatofibrosarcoma protuberans from fibrous histiocytoma in formalin-fixed, paraffin-embedded tissue. 61 54
7681857 1993
11
Oral submucosal dendrocytes: factor XIIIa+ and CD34+ dendritic cell populations in normal tissue and fibrovascular lesions. 54 61
1282135 1992
12
Fibrous histiocytoma of the tongue base. 61 54
2163249 1990
13
Primary intracranial angiomatoid fibrous histiocytoma: a case report and literature review. 61
29540076 2021
14
Reconstruction of upper limb soft-tissue defects after sarcoma resection with free flaps: A systematic review. 61
33277214 2021
15
Partial Gastrectomy at the Time of Splenectomy in Two Dogs With Splenic Neoplasia and Gastric Involvement. 61
33115688 2021
16
Cholesterotic fibrous histiocytoma: Case report and literature review. 61
33713042 2021
17
Conjunctival and Corneal Fibrous Histiocytoma: Brief review. 61
33764259 2021
18
Nonneural granular cell tumors and epithelioid fibrous histiocytoma: Similar but not the same. 61
33547837 2021
19
Risk of Rare Cancers Among Solid Organ Transplant Recipients. 61
32462187 2021
20
Dedifferentiated chondrsarcoma: a clinicopathologic analysis of 25 cases. 61
33588810 2021
21
Molecular diagnosis of an atypical case of angiomatoid fibrous histiocytoma based on detection of the EWSR1 gene translocation. 61
33624342 2021
22
Distinct clinicopathological features of pulmonary primary angiomatoid fibrous histiocytoma: A report of four new cases and review of the literature. 61
33314685 2021
23
Angiomatoid fibrous histiocytoma with Castleman disease: A case report. 61
32672859 2021
24
Secondary undifferentiated pleomorphic sarcoma of the mandible in a HIV patient who underwent radiotherapy for oral carcinoma. 61
33543781 2021
25
Broadening the spectrum of NTRK rearranged mesenchymal tumors and usefulness of pan-TRK immunohistochemistry for identification of NTRK fusions. 61
32860002 2021
26
A rare case of atrophic dermatofibroma with dermoscopic findings. 61
33403769 2021
27
Endoscopic Resection of a T-Shaped Benign Fibrous Histiocytoma at the Skull Base in a Pediatric Patient. 61
32941204 2021
28
FISH for EWSR1 in Ewing's sarcoma family of tumors: Experience from a tertiary care cancer center. 61
33433416 2021
29
Intracranial angiomatoid fibrous histiocytoma with rhabdoid features: a mimic of rhabdoid meningioma. 61
33432531 2021
30
Malignancy in giant cell tumor of bone: analysis of an open-label phase 2 study of denosumab. 61
33482769 2021
31
Cytological diagnosis of angiomatoid fibrous histiocytoma: Report of a case and review of literature. 61
32749779 2021
32
PD-L1 expression in angiomatoid fibrous histiocytoma. 61
33500467 2021
33
Paraneoplastic Secretion of Multiple Phosphatonins From a Deep Fibrous Histiocytoma Causing Oncogenic Osteomalacia. 61
33462615 2021
34
Intracranial Myxoid Mesenchymal Tumor/Myxoid Subtype Angiomatous Fibrous Histiocytoma: Diagnostic and Prognostic Challenges. 61
32970137 2020
35
Clinical outcomes of non-osteogenic, non-Ewing soft-tissue sarcoma of bone--experience of the Toronto Sarcoma Program. 61
33063945 2020
36
Angiomatoid fibrous histiocytoma in the spinal canal of T3-T4: a case report and literature review. 61
33284054 2020
37
Intracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusion. 61
33080563 2020
38
EWSR1-CREM fusion in pulmonary mesenchymal neoplasm showing distinctive clear cell morphology. 61
33002291 2020
39
Epithelioid Fibrous Histiocytoma With Dot-Like Perinuclear ALK Expression and PRKAR2A-ALK Fusion. 61
32379091 2020
40
Spindle cell variant of epithelioid cell histiocytoma (spindle cell histiocytoma) with ALK gene fusions: Cases series and review of the literature. 61
33217002 2020
41
Intranodal pressure of a metastatic lymph node reflects the response to lymphatic drug delivery system. 61
32882076 2020
42
Primary Intracranial Angiomatoid Fibrous Histiocytoma: Two Case Reports and Literature Review. 61
32777394 2020
43
Adult Intracranial Myxoid Mesenchymal Tumor with EWSR1-ATF1 Gene Fusion. 61
32683005 2020
44
Silent Angiomatoid Fibrous Histiocytoma of the Chest Wall. 61
33159863 2020
45
EWSR1/FUS-CREB fusions define a distinctive malignant epithelioid neoplasm with predilection for mesothelial-lined cavities. 61
32770123 2020
46
Intracranial mesenchymal tumor with FET-CREB fusion-A unifying diagnosis for the spectrum of intracranial myxoid mesenchymal tumors and angiomatoid fibrous histiocytoma-like neoplasms. 61
33141488 2020
47
Broadening the Age of Incidence of Intracranial Angiomatoid Fibrous Histiocytoma With EWSR1-CREM Fusion: A Case Report. 61
33085975 2020
48
Primary Pulmonary Myxoid Sarcoma and Myxoid Angiomatoid Fibrous Histiocytoma: A Unifying Continuum With Shared and Distinct Features. 61
32773530 2020
49
Feline giant-cell pleomorphic sarcoma: cytologic, histologic and immunohistochemical characterization. 61
33206030 2020
50
Successful treatment of recurrent benign fibrous histiocytoma by complete surgical removal and appropriate ocular surface reconstruction: A case report. 61
33126361 2020
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Variations for Fibrous Histiocytoma

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Expression for Fibrous Histiocytoma

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Search GEO for disease gene expression data for Fibrous Histiocytoma.
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Pathways for Fibrous Histiocytoma

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Pathways related to Fibrous Histiocytoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Transcriptional misregulation in cancer 36
11.54 MITF FUS EWSR1 ATF1
2
Striated Muscle Contraction 65 1
11.19 VIM DES ACTC1
3
Signaling mediated by p38-alpha and p38-beta 1
10.34 MITF CREB1 ATF1
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GO Terms for Fibrous Histiocytoma

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Cellular components related to Fibrous Histiocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 ATF4-CREB1 transcription factor complex GO:1990589 8.8 CREM CREB1 ATF1

Biological processes related to Fibrous Histiocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 muscle filament sliding GO:0030049 9.13 VIM DES ACTC1
2 glomerular endothelium development GO:0072011 8.62 PECAM1 CD34

Molecular functions related to Fibrous Histiocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.93 VIM SERPINA3 S100B PECAM1 MTAP MITF
2 identical protein binding GO:0042802 9.23 VIM S100B FUS EWSR1 DES CREB1
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Sources for Fibrous Histiocytoma

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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