We describe the surgical management of a rare pulmonary angiomatoid fibrous histiosarcoma (AFH). A 62-year-old lady presented with shortness of breath and found to have a large left main pulmonary artery defect that was positron emission tomography-avid. Following discussion in a thoracic multidisciplinary team meeting it was deemed unsafe to biopsy considering its intravascular position. The patient proceeded to theatre for a left pneumonectomy. She was successfully discharged home by Day 7. On follow-up the patient is well, and free of malignancy. AFH is an incredibly rare form of sarcoma, and in particular in the thoracic region. We have demonstrated successful oncological resection of a rare intravascular lesion in the pulmonary artery.
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