MCID: HST022
MIFTS: 26

Histiocytoma, Angiomatoid Fibrous

Categories: Genetic diseases

Aliases & Classifications for Histiocytoma, Angiomatoid Fibrous

MalaCards integrated aliases for Histiocytoma, Angiomatoid Fibrous:

Name: Histiocytoma, Angiomatoid Fibrous 57 73
Angiomatoid Fibrous Histiocytoma 75 29 40
Histiocytoma, Angiomatoid Fibrous, Somatic 57 13
Afh 75

Characteristics:

HPO:

32
histiocytoma, angiomatoid fibrous:
Inheritance somatic mutation


Classifications:



External Ids:

OMIM 57 612160
MedGen 42 C1266127
MeSH 44 D051677
SNOMED-CT via HPO 69 124975008 128741006 302843004
UMLS 73 C1266127

Summaries for Histiocytoma, Angiomatoid Fibrous

UniProtKB/Swiss-Prot : 75 Angiomatoid fibrous histiocytoma: A distinct variant of malignant fibrous histiocytoma that typically occurs in children and adolescents and is manifest by nodular subcutaneous growth. Characteristic microscopic features include lobulated sheets of histiocyte-like cells intimately associated with areas of hemorrhage and cystic pseudovascular spaces, as well as a striking cuffing of inflammatory cells, mimicking a lymph node metastasis.

MalaCards based summary : Histiocytoma, Angiomatoid Fibrous, also known as angiomatoid fibrous histiocytoma, is related to histiocytoma and fibrous histiocytoma. An important gene associated with Histiocytoma, Angiomatoid Fibrous is CREB1 (CAMP Responsive Element Binding Protein 1). The drugs Doxorubicin and Mechlorethamine have been mentioned in the context of this disorder. Affiliated tissues include lymph node, bone and lung, and related phenotype is histiocytoma.

Wikipedia : 76 Angiomatoid fibrous histiocytoma, abbreviated AFH, is a rarely metastasizing tumour that affects... more...

Description from OMIM: 612160

Related Diseases for Histiocytoma, Angiomatoid Fibrous

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

# Related Disease Score Top Affiliating Genes
1 histiocytoma 10.3
2 fibrous histiocytoma 10.3

Symptoms & Phenotypes for Histiocytoma, Angiomatoid Fibrous

Clinical features from OMIM:

612160

Human phenotypes related to Histiocytoma, Angiomatoid Fibrous:

32
# Description HPO Frequency HPO Source Accession
1 histiocytoma 32 HP:0012315

Drugs & Therapeutics for Histiocytoma, Angiomatoid Fibrous

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

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
2
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
3
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
4
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
5
Doxil Approved June 1999 Phase 2, Phase 3 31703
6
Isophosphamide mustard Phase 2, Phase 3 0
7 Alkylating Agents Phase 2, Phase 3
8 Topoisomerase Inhibitors Phase 2, Phase 3
9 Anti-Bacterial Agents Phase 2, Phase 3
10 Antibiotics, Antitubercular Phase 2, Phase 3
11 Antineoplastic Agents, Alkylating Phase 2, Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery Recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride

Search NIH Clinical Center for Histiocytoma, Angiomatoid Fibrous

Genetic Tests for Histiocytoma, Angiomatoid Fibrous

Genetic tests related to Histiocytoma, Angiomatoid Fibrous:

# Genetic test Affiliating Genes
1 Angiomatoid Fibrous Histiocytoma 29 CREB1

Anatomical Context for Histiocytoma, Angiomatoid Fibrous

MalaCards organs/tissues related to Histiocytoma, Angiomatoid Fibrous:

41
Lymph Node, Bone, Lung

Publications for Histiocytoma, Angiomatoid Fibrous

Articles related to Histiocytoma, Angiomatoid Fibrous:

(show top 50) (show all 67)
# Title Authors Year
1
Retroperitoneal Angiomatoid Fibrous Histiocytoma Presenting as a Recurrent Spontaneous Retroperitoneal Hemorrhage in a 9-Year-Old Boy. ( 29432314 )
2018
2
Primary intracranial angiomatoid fibrous histiocytoma: a case report and literature review. ( 29540076 )
2018
3
ALK Expression in Angiomatoid Fibrous Histiocytoma: A Potential Diagnostic Pitfall. ( 29877921 )
2018
4
Therapeutic response of metastatic angiomatoid fibrous histiocytoma carrying EWSR1-CREB1 fusion to the interleukin-6 receptor antibody tocilizumab. ( 29932283 )
2018
5
Angiomatoid Fibrous Histiocytoma With Paraneoplastic Platelet Storage Pool Deficiency. ( 29472492 )
2018
6
ESWR1-CREM Fusion in an Intracranial Myxoid Angiomatoid Fibrous Histiocytoma-Like Tumor: A Case Report and Literature Review. ( 29788195 )
2018
7
A Case of Angiomatoid Fibrous Histiocytoma Presenting as Subcutaneous Nodule in a 4-Year-Old Girl. ( 29606824 )
2018
8
TLE-1-Positive Angiomatoid Fibrous Histiocytoma Mimicking Synovial Sarcoma. ( 28248724 )
2017
9
Angiomatoid fibrous histiocytoma of the scalp mimicking cutaneous angiosarcoma in incisional biopsy containing CD31-positive histiocytes: A potential histopathological pitfall. ( 28370384 )
2017
10
CORR Insights(Ar): Pathologically Benign Lymph Nodes Can Mimic Malignancy on Imaging in Patients With Angiomatoid Fibrous Histiocytoma. ( 28656494 )
2017
11
Pathologically Benign Lymph Nodes Can Mimic Malignancy on Imaging in Patients With Angiomatoid Fibrous Histiocytoma. ( 28536856 )
2017
12
Intracranial myxoid mesenchymal tumors with EWSR1-CREB family gene fusions: myxoid variant of angiomatoid fibrous histiocytoma or novel entity? ( 28281318 )
2017
13
'Pure' spindle cell variant of angiomatoid fibrous histiocytoma, lacking classic histologic features. ( 27637157 )
2016
14
Angiomatoid fibrous histiocytoma: Clinicopathological spectrum of five cases, including EWSR1-CREB1 positive result in a single case. ( 27166030 )
2016
15
Intracranial Angiomatoid Fibrous Histiocytoma: Case Report and Literature Review. ( 27667574 )
2016
16
Angiomatoid fibrous histiocytoma: A case of local recurrence and metastases to loco-regional lymph nodes that responded to chemotherapy. ( 28012233 )
2016
17
Angiomatoid fibrous histiocytoma: novel MR imaging findings. ( 26919861 )
2016
18
Primary Angiomatoid Fibrous Histiocytoma of the Lung with Mediastinal Lymph Node Metastasis. ( 27544801 )
2016
19
Imaging of childhood angiomatoid fibrous histiocytoma with pathological correlation. ( 26162466 )
2015
20
Paraneoplastic Syndrome of Angiomatoid Fibrous Histiocytoma May Be Caused by EWSR1-CREB1 Fusion-induced Excessive Interleukin-6 Production. ( 26165403 )
2015
21
Clinicopathological features of angiomatoid fibrous histiocytoma: a series of 21 cases with variant morphology. ( 25755773 )
2015
22
Primary intracranial angiomatoid fibrous histiocytoma presenting with anaemia and migraine-like headaches and aura as early clinical features. ( 25900984 )
2015
23
Characterization of metastatic angiomatoid fibrous histiocytoma. ( 25757019 )
2015
24
Angiomatoid fibrous histiocytoma: comparison of fluorescence in situ hybridization and reverse transcription polymerase chain reaction as adjunct diagnostic modalities. ( 25804255 )
2015
25
Angiomatoid fibrous histiocytoma: clinicopathological and molecular characterisation with emphasis on variant histomorphology. ( 24043718 )
2014
26
Recurrent angiomatoid fibrous histiocytoma: a case report and review of the literature. ( 25249813 )
2014
27
Recurrent painful perianal subcutaneous angiomatoid fibrous histiocytoma: a case report and review of the literature. ( 25526437 )
2014
28
Angiomatoid Fibrous Histiocytoma With Prominent Myxoid Stroma: A Case Report and Review of the Literature. ( 25406850 )
2014
29
Solid Variant of Angiomatoid Fibrous Histiocytoma Masked by Interstitial Granuloma Annulare in a 13-year-old Child: No Evidence for Translocation Breakpoints. ( 24002209 )
2013
30
At the Intersection of Primary Pulmonary Myxoid Sarcoma and Pulmonary Angiomatoid Fibrous Histiocytoma: Observations from Three New Cases. ( 24372335 )
2013
31
Angiomatoid fibrous histiocytoma as a second tumor in a young adult with testicular cancer. ( 24155684 )
2013
32
Retroperitoneal angiomatoid fibrous histiocytoma: A case report and review of the literature. ( 23833650 )
2013
33
Metastatic angiomatoid fibrous histiocytoma of the scalp, with EWSR1-CREB1 gene fusions in primary tumor and nodal metastasis. ( 23199525 )
2013
34
Subcutaneous angiomatoid fibrous histiocytoma mimicking metastatic melanoma. ( 23320232 )
2012
35
Angiomatoid fibrous histiocytoma: pleomorphic variant associated with multiplication of EWSR1-CREB1 fusion gene. ( 22038502 )
2012
36
Angiomatoid fibrous histiocytoma a series of five cytologic cases with literature review and emphasis on diagnostic pitfalls. ( 22045622 )
2012
37
Angiomatoid fibrous histiocytoma on the hard palate: case report. ( 21965132 )
2012
38
Endobronchial pulmonary angiomatoid fibrous histiocytoma: two cases with EWSR1-CREB1 and EWSR1-ATF1 fusions. ( 22588066 )
2012
39
Angiomatoid fibrous histiocytoma: a case report and review of the literature. ( 23372863 )
2012
40
Imaging characteristics of angiomatoid fibrous histiocytoma of bone. ( 20803341 )
2011
41
Angiomatoid fibrous histiocytoma: unusual sites and unusual morphology. ( 21822206 )
2011
42
Angiomatoid fibrous histiocytoma: staging and evaluation of response to therapy with F-18 FDG PET/CT. ( 21467859 )
2011
43
Angiomatoid fibrous histiocytoma: magnetic resonance imaging appearance in 2 cases. ( 20861788 )
2010
44
An angiomatoid fibrous histiocytoma over the left pre auricular region in a 13-year-old boy. ( 20492821 )
2010
45
Angiomatoid fibrous histiocytoma including cases with pleomorphic features analysed by fluorescence in situ hybridisation. ( 20154033 )
2010
46
A rare case of cystic variety of angiomatoid fibrous histiocytoma. ( 21222377 )
2010
47
Angiomatoid fibrous histiocytoma of bone: a calcifying sclerosing variant mimicking osteosarcoma. ( 20090505 )
2010
48
Intracranial angiomatoid fibrous histiocytoma presenting as recurrent multifocal intraparenchymal hemorrhage. ( 19731989 )
2010
49
Angiomatoid fibrous histiocytoma in a 25-year-old male. ( 21139823 )
2010
50
Utility of FISH in the diagnosis of angiomatoid fibrous histiocytoma: a series of 18 cases. ( 19801966 )
2010

Variations for Histiocytoma, Angiomatoid Fibrous

Expression for Histiocytoma, Angiomatoid Fibrous

Search GEO for disease gene expression data for Histiocytoma, Angiomatoid Fibrous.

Pathways for Histiocytoma, Angiomatoid Fibrous

GO Terms for Histiocytoma, Angiomatoid Fibrous

Sources for Histiocytoma, Angiomatoid Fibrous

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69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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