Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumour that rarely behaves malignant. We report a radical resection of a mediastinal angiomatoid fibrous histiocytoma, which grew invasively into the pulmonary artery wall, was adherent to the posterior aorta and close to the main stem of the left coronary artery. A transection of the aorta was performed using cardiopulmonary bypass and cardioplegic arrest for a safe and radical removal that resulted in symptom relief.

BACKGROUND

Angiomatoid fibrous histiocytoma (AFH) is a rare, low-grade malignant soft tissue neoplasm that presents distinctive diagnostic dilemmas due to its clinical and radiological resemblance to various malignancies [1, 2]. While typically a slow-growing tumour found in the extremities of children and young adults, extra-somatic soft tissue sites of AFH, like the mediastinum, have a higher mean age of 35 at presentation and are more frequently associated with systemic symptoms such as pyrexia, anaemia and malaise [3].

Here, we describe a challenging but successful resection of a deep mediastinal AFH located behind the aortic root with invasion of the right pulmonary artery. Due to the rarity and surgical challenges of mediastinal AFH, this case highlights the need for a multidisciplinary approach, including cardiopulmonary bypass.