Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue
cancer
Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...
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.
Pathology
It is characterized by cystic blood-filled spaces and composed of
histiocyte
A histiocyte is a vertebrate cell that is part of the mononuclear phagocyte system (also known as the reticuloendothelial system or lymphoreticular system). The mononuclear phagocytic system is part of the organism's immune system. The histiocyt ...
-like cells. A
lymphocytic cuff is common. It often simulates a vascular lesion, and was initially described as doing this.
AFH typically has a
chromosomal translocation
In genetics, chromosome translocation is a phenomenon that results in unusual rearrangement of chromosomes. This includes balanced and unbalanced translocation, with two main types: reciprocal-, and Robertsonian translocation. Reciprocal transl ...
involving the
ATF1 gene -- t(12;16) FUS/ATF1 or t(12;22) EWS/ATF1.
Diagnosis
Treatment
See also
*
Granulomatous inflammation
*
Vascular tumor
A vascular tumor is a tumor of vascular origin; a soft tissue growth that can be either benign or malignant, formed from blood vessels or lymph vessels. Examples of vascular tumors include hemangiomas, lymphangiomas, hemangioendotheliomas, K ...
References
External links
Soft tissue disorders
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