Angiomatoid Fibrous Histiocytoma First Report of Primary Pulmonary Origin
Angiomatoid Fibrous Histiocytoma First Report of Primary Pulmonary Origin
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DOI：
10.1097/pas.0b013e3181b20e64
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发表时间：
2009-10-01
影响因子：
5.6
通讯作者：
Chan, John K. C.
中科院分区：
文献类型：
--
作者：
Ren, Li;Guo, Shuang-Ping;Chan, John K. C.
血管瘤样纤维组织细胞瘤是一种少见的软组织肿瘤，最常累及儿童和青壮年的真皮深层和皮下组织。我们报告第一个病例表现为原发肺部肿瘤，患者为46岁男性。组织学上。肿瘤由多个细胞结节组成，周围有纤维性假包膜和瘤周淋巴浆细胞浸润物。结节由组织细胞样细胞组成，呈弥漫的、轮生的或模糊的条状图案，其间密布浆细胞和淋巴细胞。肿瘤细胞上皮膜抗原呈阳性反应，结蛋白、CD68、CD163呈灶性阳性。荧光原位杂交显示EWS基因易位，经聚合酶链式反应进一步证实为EWS/atf1基因融合所致。重要的是要认识到，血管瘤样纤维组织细胞瘤可以发生在肺部，因为它的组织学特征很难描述，因此可能被误认为其他肿瘤，如脑膜瘤、炎性肌纤维母细胞瘤和滤泡性树突状细胞肉瘤。
Angiomatoid fibrous histiocytoma is all 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.