AFH
MCID: HST022
MIFTS: 26

Histiocytoma, Angiomatoid Fibrous (AFH)

Categories: Cancer diseases, 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 Cyclophosphamide 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:

(show all 12)
# Related Disease Score Top Affiliating Genes
1 histiocytoma 10.5
2 fibrous histiocytoma 10.5
3 sarcoma 10.3
4 storage pool platelet disease 10.2
5 sarcoma, synovial 10.2
6 membranous nephropathy 10.2
7 polyarteritis nodosa, childhood-onset 10.2
8 granuloma annulare 10.2
9 angiosarcoma 10.2
10 melanoma 10.2
11 testicular cancer 10.2
12 polyarteritis nodosa 10.2

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



Diseases related to Histiocytoma, Angiomatoid Fibrous

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
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 6055-19-2, 50-18-0 2907
2
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
3
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
4
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
5
Doxil Approved June 1999 Phase 2, Phase 3 31703
6
Isophosphamide mustard Phase 2, Phase 3 0
7 Anti-Bacterial Agents Phase 2, Phase 3
8 Antineoplastic Agents, Alkylating Phase 2, Phase 3
9 Antibiotics, Antitubercular Phase 2, Phase 3
10 Alkylating Agents Phase 2, Phase 3
11 Topoisomerase Inhibitors 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 Suspended NCT02180867 Phase 2, Phase 3 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride
2 Angiomatoid Fibrous Histiocytoma: a Single Institution Case-series Not yet recruiting NCT03759327

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, Skin

Publications for Histiocytoma, Angiomatoid Fibrous

Articles related to Histiocytoma, Angiomatoid Fibrous:

(show top 50) (show all 87)
# 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
Treatment of angiomatoid fibrous histiocytoma after unplanned excision: a case report. ( 30170609 )
2018
9
TLE-1-Positive Angiomatoid Fibrous Histiocytoma Mimicking Synovial Sarcoma. ( 28248724 )
2017
10
Angiomatoid fibrous histiocytoma of the scalp mimicking cutaneous angiosarcoma in incisional biopsy containing CD31-positive histiocytes: A potential histopathological pitfall. ( 28370384 )
2017
11
CORR Insights(Ar): Pathologically Benign Lymph Nodes Can Mimic Malignancy on Imaging in Patients With Angiomatoid Fibrous Histiocytoma. ( 28656494 )
2017
12
Pathologically Benign Lymph Nodes Can Mimic Malignancy on Imaging in Patients With Angiomatoid Fibrous Histiocytoma. ( 28536856 )
2017
13
Intracranial myxoid mesenchymal tumors with EWSR1-CREB family gene fusions: myxoid variant of angiomatoid fibrous histiocytoma or novel entity? ( 28281318 )
2017
14
Angiomatoid fibrous histiocytoma: a series of seven cases including genetically confirmed aggressive cases and a literature review. ( 28114920 )
2017
15
'Pure' spindle cell variant of angiomatoid fibrous histiocytoma, lacking classic histologic features. ( 27637157 )
2016
16
Angiomatoid fibrous histiocytoma: Clinicopathological spectrum of five cases, including EWSR1-CREB1 positive result in a single case. ( 27166030 )
2016
17
Intracranial Angiomatoid Fibrous Histiocytoma: Case Report and Literature Review. ( 27667574 )
2016
18
Angiomatoid fibrous histiocytoma: A case of local recurrence and metastases to loco-regional lymph nodes that responded to chemotherapy. ( 28012233 )
2016
19
Angiomatoid fibrous histiocytoma: novel MR imaging findings. ( 26919861 )
2016
20
Primary Angiomatoid Fibrous Histiocytoma of the Lung with Mediastinal Lymph Node Metastasis. ( 27544801 )
2016
21
Imaging of childhood angiomatoid fibrous histiocytoma with pathological correlation. ( 26162466 )
2015
22
Paraneoplastic Syndrome of Angiomatoid Fibrous Histiocytoma May Be Caused by EWSR1-CREB1 Fusion-induced Excessive Interleukin-6 Production. ( 26165403 )
2015
23
Clinicopathological features of angiomatoid fibrous histiocytoma: a series of 21 cases with variant morphology. ( 25755773 )
2015
24
Primary intracranial angiomatoid fibrous histiocytoma presenting with anaemia and migraine-like headaches and aura as early clinical features. ( 25900984 )
2015
25
Characterization of metastatic angiomatoid fibrous histiocytoma. ( 25757019 )
2015
26
Angiomatoid fibrous histiocytoma: comparison of fluorescence in situ hybridization and reverse transcription polymerase chain reaction as adjunct diagnostic modalities. ( 25804255 )
2015
27
Angiomatoid fibrous histiocytoma - case series with emphasis on a late fibrotic variant. ( 25918088 )
2015
28
Angiomatoid fibrous histiocytoma: the current status of pathology and genetics. ( 25927151 )
2015
29
Angiomatoid fibrous histiocytoma: case report and review of the literature. ( 26613231 )
2015
30
Angiomatoid Fibrous Histiocytoma With Prominent Myxoid Stroma: A Case Report and Review of the Literature. ( 25406850 )
2015
31
Angiomatoid fibrous histiocytoma: clinicopathological and molecular characterisation with emphasis on variant histomorphology. ( 24043718 )
2014
32
Recurrent angiomatoid fibrous histiocytoma: a case report and review of the literature. ( 25249813 )
2014
33
Recurrent painful perianal subcutaneous angiomatoid fibrous histiocytoma: a case report and review of the literature. ( 25526437 )
2014
34
A case report: angiomatoid fibrous histiocytoma in a 6-year-old male and review of the literature. ( 24521435 )
2014
35
Angiomatoid fibrous histiocytoma: an expansion of the clinical and histological spectrum. ( 24614712 )
2014
36
Angiomatoid fibrous histiocytoma of the pulmonary artery: a multidisciplinary discussion. ( 24702653 )
2014
37
Ultrasound B-mode and elastographic findings of angiomatoid fibrous histiocytoma. ( 24758358 )
2014
38
Membranous nephropathy associated with angiomatoid fibrous histiocytoma in a pediatric patient. ( 24880818 )
2014
39
Solid Variant of Angiomatoid Fibrous Histiocytoma Masked by Interstitial Granuloma Annulare in a 13-year-old Child: No Evidence for Translocation Breakpoints. ( 24002209 )
2013
40
At the Intersection of Primary Pulmonary Myxoid Sarcoma and Pulmonary Angiomatoid Fibrous Histiocytoma: Observations from Three New Cases. ( 24372335 )
2013
41
Angiomatoid fibrous histiocytoma as a second tumor in a young adult with testicular cancer. ( 24155684 )
2013
42
Retroperitoneal angiomatoid fibrous histiocytoma: A case report and review of the literature. ( 23833650 )
2013
43
Metastatic angiomatoid fibrous histiocytoma of the scalp, with EWSR1-CREB1 gene fusions in primary tumor and nodal metastasis. ( 23199525 )
2013
44
Subcutaneous angiomatoid fibrous histiocytoma mimicking metastatic melanoma. ( 23320232 )
2012
45
Angiomatoid fibrous histiocytoma: pleomorphic variant associated with multiplication of EWSR1-CREB1 fusion gene. ( 22038502 )
2012
46
Angiomatoid fibrous histiocytoma a series of five cytologic cases with literature review and emphasis on diagnostic pitfalls. ( 22045622 )
2012
47
Angiomatoid fibrous histiocytoma on the hard palate: case report. ( 21965132 )
2012
48
Endobronchial pulmonary angiomatoid fibrous histiocytoma: two cases with EWSR1-CREB1 and EWSR1-ATF1 fusions. ( 22588066 )
2012
49
Angiomatoid fibrous histiocytoma: a case report and review of the literature. ( 23372863 )
2012
50
Imaging characteristics of angiomatoid fibrous histiocytoma of bone. ( 20803341 )
2011

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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74 UMLS via Orphanet
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