(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
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
histological features (lymphocytic cuff, dense hyalinefibrous
pseudocapsule, pseudovascular spaces and
spaces lined with flattened tumour cells,
haemorrhage, haemosiderin, and a round or spindle fibrohistiocytic
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-CREB1 fusion genes.
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