Gong Q X, Zhang Z H, Fan Q H
Department of Pathology, First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
Zhonghua Bing Li Xue Za Zhi. 2018 Sep 8;47(9):700-705. doi: 10.3760/cma.j.issn.0529-5807.2018.09.010.
To study clinicopathologic features, diagnosis and differential diagnosis of myxoid variant of angiomatoid fibrous histiocytoma (AFH). Three cases of myxoid variant of AFHs were collected from First Affiliated Hospital of Nanjing Medical University during 2008 and 2017. EnVision method and fluorescence in situ hybridization(FISH) were used to detect immunophenotype and EWSR1 gene rearrangement, respectively. There were 2 males and l female with age at 13, 31, and 42 years, respectively. The patients presented with a painless mass located superficially (subcutaneous or submucosal) in two cases or deep-seated (retroperitoneum) in one case. Grossly, the diameters of tumors were 1, 7, and 2 cm, respectively. The cut surface was solid and firm, tan to gray in colour. Histologically, the circumscribed tumor had fibrous pseudocapsule and peritumoal lymphoplasmacytic infiltrates. The tumor cells arranged in vaguely nodular growth pattern, with prominent myxoid stroma (present in 60% to 100% of the entire tumor). In hypocellular myxoid areas, the spindle to stellate tumor cells arranged in cords or reticular pattern, or in a haphazard manner. However, histiocytoid cells arranged in fascicular, sheet-like, or whorled growth pattern, as in classical AFH, were also identified in hypercelluar areas. Mild to moderate atypia was observed with low mitotic rate of (0-2)/10 HPF. Tumor necrosis was not seen. One case presented with slit-like hemorrhage and sclerosing collagen intermingled with myxoid matrix was identified in 1 case. Immunohistochemically, all cases were positive for CD68 and CD163. Two of three were positive for desmin, EMA, CD99 and one for Calponin, SMA. All cases were negative for S-100 protein, CD34, CD31, CD35, CD21 and CKpan. FISH detection was positive for EWSRl gene in all cases. Available clinical follow-up was obtained in 2 cases, revealing no evidence of disease in 6 and 89 months, respectively. Myxoid variant of AFH is a histological subtype of AFH, with clinical features, immunophenotypes, genomic profiles and biological behavior similar to typical AFH. Their unusual morphology is easily confused with a variety of other myxoid mesenchymal neoplasms, including myoepithelioma and nerve sheath tumors.
研究血管样纤维组织细胞瘤（AFH）黏液样变型的临床病理特征、诊断及鉴别诊断。收集南京医科大学第一附属医院2008年至2017年间3例AFH黏液样变型病例。分别采用EnVision法和荧光原位杂交（FISH）检测免疫表型和EWSR1基因重排。患者2例男性，1例女性，年龄分别为13岁、31岁和42岁。2例患者表现为浅表（皮下或黏膜下）无痛性肿块，1例表现为深部（腹膜后）肿块。大体上，肿瘤直径分别为1 cm、7 cm和2 cm。切面实性、质硬，颜色为黄褐色至灰白色。组织学上，边界清楚的肿瘤有纤维性假包膜及肿瘤周围淋巴浆细胞浸润。肿瘤细胞呈模糊的结节状生长模式，有明显的黏液样间质（占整个肿瘤的60%至100%）。在细胞稀少的黏液样区域，梭形至星状肿瘤细胞呈条索状、网状排列或排列杂乱。然而，在细胞丰富区域也可见到如经典AFH中那样呈束状、片状或漩涡状生长模式的组织细胞样细胞。可见轻度至中度异型性，有丝分裂率低（0 - 2）/10个高倍视野。未见肿瘤坏死。1例可见裂隙状出血，1例可见硬化性胶原与黏液样基质混合。免疫组化显示，所有病例CD68和CD163均阳性。3例中有2例结蛋白、上皮膜抗原（EMA）、CD99阳性，1例平滑肌肌动蛋白（SMA）、钙结合蛋白阳性。所有病例S - 100蛋白、CD34、CD31、CD35、CD21和细胞角蛋白（CKpan）均阴性。FISH检测所有病例EWSR1基因均阳性。2例患者有可用的临床随访资料，分别在6个月和89个月时未发现疾病证据。AFH黏液样变型是AFH的一种组织学亚型，其临床特征、免疫表型、基因组特征及生物学行为与典型AFH相似。其不寻常的形态容易与多种其他黏液样间叶性肿瘤混淆，包括肌上皮瘤和神经鞘瘤。
