Hou Y Y, Tan Y S, Xu J F, Wang X N, Lu S H, Ji Y, Wang J, Zhu X Z
Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China.
Histopathology. 2006 Apr;48(5):536-45. doi: 10.1111/j.1365-2559.2006.02370.x.
Thirty-three cases of gastrointestinal schwannomas were analysed to elucidate their peculiar clinicopathological, immunohistochemical and ultrastructural features.
The patients were 16 men and 17 women, whose ages ranged from 27 to 81 years (median 52.6 years). Tumour size ranged from 10 to 120 mm in diameter. Follow-up in 23 cases from 6 months to 13 years showed no recurrences or metastases. Microscopically, all tumours were composed of spindle cells with focal epithelioid cells in four cases. In all cases except one, there were peripheral cuff-like lymphoid aggregates. Immunohistochemically, tumours were strongly positive for S100 protein and vimentin, the tumours were variably positive for nestin (78.8%, 26/33) and glial fibrillary acidic protein (63.6%, 21/33), three tumours had CD34+ cells, but all were negative for CD117, alpha-smooth muscle actin and desmin. Ultrastructurally, the tumours were composed of elongated spindle shaped cells with prominent parallel membranous structures.
Gastrointestinal schwannomas have characteristic histological features, especially the presence of a lymphoid cuff, that are different from their soft tissue and central nervous system counterparts. Gastrointestinal tract schwannomas behave in a benign fashion.
分析33例胃肠道神经鞘瘤,以阐明其独特的临床病理、免疫组化及超微结构特征。
患者中男性16例,女性17例,年龄范围为27至81岁(中位年龄52.6岁)。肿瘤直径为10至120mm。23例患者随访6个月至13年,未见复发或转移。镜下,所有肿瘤均由梭形细胞组成,4例可见局灶性上皮样细胞。除1例之外,所有病例均可见外周袖套样淋巴样聚集。免疫组化结果显示,肿瘤S100蛋白和波形蛋白呈强阳性,巢蛋白(78.8%,26/33)和胶质纤维酸性蛋白(63.6%,21/33)呈不同程度阳性,3例肿瘤有CD34+细胞,但CD117、α平滑肌肌动蛋白和结蛋白均为阴性。超微结构上,肿瘤由细长的梭形细胞组成,具有明显的平行膜性结构。
胃肠道神经鞘瘤具有特征性组织学特征,尤其是存在淋巴样袖套,这与软组织和中枢神经系统的神经鞘瘤不同。胃肠道神经鞘瘤表现为良性。