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Pathology of Angiomatoid Fibrous Histiocytoma 


Dr Sampurna Roy MD   


Angiomatoid Fibrous Histiocytoma - [Pathology Infographic]




(1) Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumour, with a low-grade malignant potential and unknown histogenesis.

(2) This tumour predominantly occurs in the deep dermis and subcutis of the extremities  of children and young adults.  

(3) According to the World Health Organization (WHO) Classification of Tumours of Soft Tissue and Bone, Angiomatoid fibrous histiocytoma (AFH) is described as an intermediate soft-tissue tumour that is often locally aggressive. 

(4) In 1979 Enzinger first described it as a distinct fibrohistiocytic tumour of children and young adults, simulating a vascular neoplasm according to 41 examples of an "unusual fibrohistiocytic sarcoma". He called it  "angiomatoid malignant fibrous histiocytoma."

(5) The tumour was later formally renamed AFH because of its slow growth and rare metastasis according to WHO classification of soft-tissue tumors ( 2002 ).

(6) Although AFH is most commonly associated with the extremities, there have been a number of reported cases of AFH arising at additional sites such as the mediastinum, bone, intracranial tissue, retroperitoneum, lung, vulva, and ovary.

(7) About two-thirds of cases occur at sites with abundant lymph nodes, such as the antecubital fossa, popliteal fossa, axilla, and inguinal and supraclavicular areas.

(8) AFH is often associated with systemic symptoms such as anemia, fever, weight loss, enhanced cytokine production, polyclonal gammopathy, and a Castleman disease-like lymphadenopathy.

(9)  Classical histological features (lymphocytic cuff, dense hyalinefibrous pseudocapsule, pseudovascular spaces and blood-filled cystic spaces lined with flattened tumour cells, haemorrhage, haemosiderin, and a round or spindle fibrohistiocytic cell proliferation.

(10) Interpreting immunohistochemical staining results can be complex, as there is not one immunohistochemical marker that consequently stains positive for AFH. 

The tumour is often positive for CD68, CD99, epithelial membrane antigen (EMA), and desmin, and negative for endothelial markers, such as factor VIII-related antigen and CD34.

(11) Cytogenetic and molecular studies in AFH have reported three specific gene rearrangements, involving the FUS-ATF1, EWSR1-ATF1, or EWSR1-CREB1 fusion genes.

(12) AFH can recur locally, but most of these patients do well with wide local excisions alone, if a wide surgical margin is possible.

Radiotherapy may be utilized when wide excision margins are not feasible.

AFH does have low potential for metastasis, but surgery can be effective in these cases as well.







Lee H-S, Kim T, Kim J-S, et al. Angiomatoid Fibrous Histiocytoma as a Second Tumor in a Young Adult with Testicular Cancer. Cancer Research and Treatment: Official Journal of Korean Cancer Association. 2013;45(3):239-243


Mansfield A, Larson B, Stafford SL, Shives TC, Haddock MG, Dingli D. Angiomatoid fibrous histiocytoma in a 25-year-old male. Rare Tumors. 2010;2(2):e20.


Bauer A, Jackson B, Marner E, Gilbertson-Dahdal D. Angiomatoid Fibrous Histiocytoma: A Case Report and Review of the Literature. Journal of Radiology Case Reports. 2012;6(11):8-15.


Kong X, Zhao D, Lin G, Zhou J, Cui Q. Recurrent Painful Perianal Subcutaneous Angiomatoid Fibrous Histiocytoma: A Case Report and Review of the Literature. Gosain. A, ed. Medicine. 2014;93(28):e202.


Enzinger FM. Angiomatoid malignant fibrous histiocytoma. A distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. Cancer. 1979;44 (6) 2147 - 2157


Weiss S, Goldblum J. Soft Tissue Tumors. Elsevier; 2008










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