Angiomatoid fibrous histiocytoma in a six-year-old child

We report a case of AFH presenting as an asymptomatic subcutaneous nodule on the arm of a 6-year-old boy. AFH is a fibrohistiocytic tumor of intermediate malignancy. Predominantly seen in children and young adults, AFH presents as a deep dermal or subcutaneous nodule usually on the extremities. The histology is characterized by a fibrous capsule, surrounding lymphocytic infiltrate and blood-filled cystic spaces lined by flattened tumor cells. AFH cells express desmin, epithelial membrane antigen, and CD 68 in over 60% of cases; they are negative for myogenin, MYOD1, and endothelial markers. Rate of local recurrence is 2% to 20%. The metastatic rate is 1%. Management is with wide surgical excision and careful follow-up.