Department of General Surgery, Aizawa Hospital, Matsumoto, Japan.
Department of Anatomic Pathology, Aizawa Hospital, Matsumoto, Japan.
Thorac Cancer. 2021 May;12(9):1453-1456. doi: 10.1111/1759-7714.13929. Epub 2021 Mar 13.
Angiomatoid fibrosis histiocytoma (AFH) is a rare neoplastic disease. Only one report has demonstrated an intraluminal tumor of the pulmonary artery (PA) corresponding to AFH to date. We describe the case of AFH with EWSR1-CREB1 fusion occurring in the ascending artery. A 42-year-old man exhibited an abnormal nodule on chest computed tomography (CT) during checkup. It revealed an intraluminal mass in the ascending artery with significant metabolic uptake in positron emission tomography (PET)/CT. Therefore, right upper lobectomy with wedge resection of the PA trunk was performed. Histologically, the tumor was multinodular and surrounded by a dense lymphoplasmacytic cuff. Each nodule was composed of myxoid stroma and comprised ovoid or spindle cell fascicles with mild atypia. Fluorescent in situ hybridization (FISH) analysis confirmed EWSR1-CREB1 fusion. A diagnosed as AFH was made. This report widens the spectrum of differential diagnoses of primary tumors occurring in the PA.
血管肌纤维母细胞瘤（angiomatoid fibromyxoid histiocytoma，AFH）是一种罕见的肿瘤性疾病。迄今为止，仅有一份报告显示肺动脉（PA）内存在与 AFH 相对应的腔内肿瘤。我们描述了一例发生于升主动脉的 EWSR1-CREB1 融合的 AFH 病例。一名 42 岁男性在体检时胸部计算机断层扫描（CT）发现异常结节。PET/CT 显示升主动脉内有腔内肿块，伴有明显代谢摄取。因此，进行了右上肺叶切除术和 PA 干楔形切除术。组织学上，肿瘤呈多结节状，周围有致密的淋巴浆细胞袖套。每个结节由黏液样基质组成，包含卵圆形或梭形细胞束，轻度异型性。荧光原位杂交（fluorescent in situ hybridization，FISH）分析证实存在 EWSR1-CREB1 融合。诊断为 AFH。本报告拓宽了 PA 内原发性肿瘤的鉴别诊断范围。
