Angiomatoid Fibrous Histiocytoma: A Rare Entity With Recognized Diagnostic Pitfalls

Angiomatoid Fibrous Histiocytoma (AFH) is a rare soft tissue tumour of indeterminate differentiation usually occurring in children and young adults with low rate of metastasis and recurrence. AFH continues to be revised with various studies reporting recurrences but up till now, the diagnostic criterion remains unclear with pathologists relying mainly on Enzinger’s description and immunohistochemistry. Adding to the fact that it is a rare tumour, diagnosis usually is delayed. We present a case of a 10 year old male with AFH with atypical histology, immunoprofile and genetic testing. Opinions were sought from Heidelberg hospital Germany and Children Hospital of Philadelphia which had contrasting views regarding the diagnosis, which was finally confirmed by Brigham and Women’s hospital, Boston. We review literature and discuss the possible differential diagnosis.