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.
Keywords: Angiomatoid fibrous histiocytoma; EWSR1; Fluorescence in situ hybridization; Pulmonary neoplasms; Soft tissue neoplasms.
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