Angiomatoid Fibrous Histiocytoma: A Rare Cause of Anemia in a Child

Abstract

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of intermediate potential and is usually found during first two decades of life. It is located mainly in extremities presents as a painless, slow growing mass with extensive blood supply. Embryologically, it arises from a pluripotent mesenchymal cell. Histologically, the tumor has thick pseudo capsule with chronic inflammatory infiltrates and hemorrhagic cystic areas. We present a case of AFH which caused severe anemia in a child with a non-healing ulcer on the medial aspect of left knee. Multiple blood transfusions, incision and drainage and biopsies were done however; only complete surgical resection proved to be fruitful. The biopsy is EMA positive, cytokeratin AE1/AE3 & Desmin are focal positive. CD21 highlights meshwork of follicular dendritic cells. Yearly follow up is advised. Angiomatoid Fibrous Histiocytoma should be kept in the differentials while managing hematoma-like lesions in the extremities. It is often misdiagnosed thus prompt treatment is usually delayed. The prognosis after complete surgical resection is good but chemotherapy should be considered in recurrent cases.