Keloidal atypical fibroxanthoma of the scalp

Atypical fibroxanthoma (AFX) is a rare low-grade sarcoma that occurs mainly in the elderly and may recur locally. There are multiple variants including keloidal AFX (KAF). KAF is characterised by a proliferation of atypical spindled and epithelioid cells admixed with bizarre pleomorphic cells. These cells intersect among broad bands of keloidal collagen. AFX is a diagnosis of exclusion so a broad panel of immunohistochemical staining should be applied to rule out other differentials (squamous cell carcinoma (SCC), melanoma, leiomyosarcoma, etc). There is added difficulty with the diagnosis of KAF as it may mimic multiple keloidal lesions, including exuberant scarring, as in this case. p53 immunohistochemistry staining can be useful in highlighting the presence of tumour cells. Additionally, next generation sequencing can detect genetic mutations identified in clonal proliferations consistent with tumour formation. KAF is easily overlooked and it should be included in the differential diagnosis for keloidal lesions showing even mild atypia.