Department of Pathology, Air Force General Hospital, Beijing, SAR China.
Am J Surg Pathol. 2009 Oct;33(10):1570-4. doi: 10.1097/PAS.0b013e3181b20e64.
Angiomatoid fibrous histiocytoma is an uncommon soft tissue tumor most frequently affecting the deep dermis and subcutis of the extremities in children and young adults. We report the first case presenting as a primary pulmonary tumor in a 46-year-old man. Histologically, the tumor was composed of multiple cellular nodules surrounded by a fibrous pseudocapsule and peritumoral lymphoplasmacytic infiltrates. The nodules were composed of histiocytoid cells with a diffuse, whorled, or vague storiform pattern, with the intervening areas densely packed with plasma cells and lymphocytes. The tumor cells were immunoreactive for epithelial membrane antigen, and focally desmin, CD68, and CD163. Fluorescence in-situ hybridization revealed EWS gene translocation, which was further confirmed on polymerase chain reaction to result from EWS/ATF1 gene fusion. It is important to recognize that angiomatoid fibrous histiocytoma can occur in the lung because its histologic features are rather nondescript and thus can be mistaken for other tumors such as meningioma, inflammatory myofibroblastic tumor, and follicular dendritic cell sarcoma.
血管外皮细胞瘤样纤维组织细胞瘤是一种不常见的软组织肿瘤，最常发生于儿童和青年的四肢深部真皮和皮下组织。我们报告了首例发生于 46 岁男性的原发性肺肿瘤病例。组织学上，肿瘤由多个细胞性结节组成，周围有纤维假包膜和肿瘤周围淋巴浆细胞浸润。结节由组织细胞样细胞组成，呈弥漫性、旋涡状或模糊的席纹状模式，其间区域密集排列着浆细胞和淋巴细胞。肿瘤细胞免疫组化表达上皮膜抗原，局灶性表达结蛋白、CD68 和 CD163。荧光原位杂交显示 EWS 基因易位，经聚合酶链反应进一步证实为 EWS/ATF1 基因融合。重要的是要认识到血管外皮细胞瘤样纤维组织细胞瘤可以发生在肺部，因为其组织学特征相当不典型，因此可能误诊为其他肿瘤，如脑膜瘤、炎性肌纤维母细胞瘤和滤泡树突状细胞肉瘤。
