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目的探讨腹膜胶质瘤病(GP)的发生、临床病理特征及预后。方法对1例GP进行组织形态学、免疫组化分析,并复习相关国内、外文献。结果患者女性,10岁。腹胀2个月。CT示中腹部双肾前见一类圆形巨大混杂性肿块影。术中见肿瘤21cm×18cm×7cm大小,包膜完整,表面被覆大网膜,肿物及大网膜表面满布直径0.1～0.5cm的灰白色小结节。镜下肿物由成熟三胚层组织组成,含大量神经胶质组织。大网膜和肿瘤包膜上密集的粟粒样结节,镜下均为成熟神经胶质组织,边界清楚,无浸润。免疫组化:腹膜神经胶质结节GFAP、S-100和vimentin(+),结节表面被覆的间皮细胞CK(+)。结论GP是一种罕见病症,大部分对预后无不良影响,但可有恶变,需长期随访。组织起源尚需大宗研究。
Objective To investigate the occurrence, clinicopathological features and prognosis of peritoneal gliomatosis (GP). Methods 1 case of GP histomorphology, immunohistochemical analysis, and review the relevant domestic and foreign literature. Results Female patient, 10 years old. Bloating 2 months. CT showed a large amount of circular large mixed mass before the kidney of the abdomen. See the tumor surgery 21cm × 18cm × 7cm size, complete capsule, the surface covered omentum, omentum and omentum surface covered with a diameter of 0.1 ~ 0.5cm small white nodules. Microscopic tumor consists of mature three germ layers, with a large number of glial tissue. Omental and tumor capsule dense miliary nodules, microscopic are mature glial tissue, clear boundary, no infiltration. Immunohistochemistry: peritoneal glial nodules GFAP, S-100 and vimentin (+), nodular surface covered mesothelial cells CK (+). Conclusion GP is a rare condition, most of which have no adverse effect on prognosis, but may have malignant transformation and require long-term follow-up. Organizational origin still need to bulk research.