View source for Medicine:Angiomatoid fibrous histiocytoma

{{short description|Type of tumor which affects children and adolescents}}
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| caption         = [[Micrograph]] showing an angiomatoid fibrous histiocytoma. H&E stain.
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'''Angiomatoid fibrous histiocytoma''' '''(AFH)''' is a rare soft tissue [[Medicine:Cancer|cancer]] that affects children and young adults. On November 16, 2020, US MasterChef Junior participant Ben Watkins died from the disease at the age of 14.<ref name="Ben">{{cite web |title=Ben Watkins: US MasterChef Junior star dies aged 14 |url=https://www.bbc.co.uk/news/entertainment-arts-54984423 |website=BBC News |date=18 November 2020}}</ref>

==Pathology==
It is characterized by cystic blood-filled spaces and composed of [[Biology:Histiocyte|histiocyte]]-like cells.  A [[Biology:Lymphocyte|lymphocytic cuff]] is common.  It often simulates a vascular lesion, and was initially described as doing this.<ref name=pmid228836>{{Cite journal  | last1 = Enzinger | first1 = FM. | title = Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. | journal = Cancer | volume = 44 | issue = 6 | pages = 2147–57 |date=Dec 1979 | doi =  10.1002/1097-0142(197912)44:6<2147::aid-cncr2820440627>3.0.co;2-8| pmid = 228836 }}</ref>

AFH typically has a [[Biology:Chromosomal translocation|chromosomal translocation]] involving the [[Biology:ATF1|ATF1 gene]] -- t(12;16) FUS/ATF1 or t(12;22) EWS/ATF1.<ref>{{Cite journal|last=Thway|first=Khin|last2=Fisher|first2=Cyril|date=2015-05-01|title=Angiomatoid Fibrous Histiocytoma: The Current Status of Pathology and Genetics|url=https://meridian.allenpress.com/aplm/article/139/5/674/193796/Angiomatoid-Fibrous-Histiocytoma-The-Current|journal=Archives of Pathology & Laboratory Medicine|language=en|volume=139|issue=5|pages=674–682|doi=10.5858/arpa.2014-0234-RA|issn=0003-9985|pmid=25927151|quote=Further to the characterization of the recurrent chromosomal rearrangements that result in the EWSR1-CREB1, t(12;22)(q13;q12) EWSR1-ATF1, and t(12;16)(q13;p11) FUS-ATF1 gene fusions, AFH is of course now established as a translocation-associated neoplasm.|doi-access=free}}</ref>

==Diagnosis==
==Treatment==

==See also==
* Granulomatous inflammation
* [[Medicine:Vascular tumor|Vascular tumor]]

==References==
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== External links ==
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[[Category:Soft tissue disorders]]

{{Sourceattribution|Angiomatoid fibrous histiocytoma}}