Retroperitoneal angiomatoid fibrous histiocytoma; an extremely rare entity

Angiomatoid fibrous histiocytoma (AFH) is a rare, soft tissue tumour with malignant potential that occurs most commonly in children and young adults. Only a few case reports had described this disease, which typically occurs in the extremities, trunk, head and neck or rarely in the bones. We represent an extremely rare case of a young male with retroperitoneal AFH. We emphasize that AFH may occur in the retroperitoneum so; in any patient with a retroperitoneal tumour the possibility of having AFH should be considered. Also, we highlight that surgery remains the mainstay management that can effectively control local symptoms, although complete excision is usually difficult.