Primary angiomatoid fibrous histiocytoma of the lung with mediastinal lymph node metastasis

Angiomatoid fibrous histiocytoma (AFH), a rare soft tissue tumor of borderline malignancy, typically occurs in the subcutis of the accessible soft tissues, most often in children and young adults. Lymph node and/or distant metastasis is seen in less than 2% of patients with AFH. Exceptionally rare examples of AFH have been reported in visceral locations, including the lung. We report a genetically confirmed primary pulmonary AFH in a 70-year-old woman with mediastinal lymph node metastasis, representing to the best of our knowledge the first report of metastases from a visceral AFH.