Isimbaldi G, Galli C, Declich P
Unità operativa di anatomia ed istologia patologica, Ospedale di Legnano USSL 70.
Pathologica. 1992 Jul-Aug;84(1092):557-61.
A six months female infant was admitted in our hospital for congenital dysmorphism of face: a subcutaneous nodule in left nose region was present. An x-ray study showed relevant scoliosis of the nasal septum. On surgery a white firm nodule was incompletely excised; a post-operatory CT-scan excluded any communication of neoplasia with brain. No bone lacunae were seen. Clinically there was neither rhinorrhea nor meningitis. The baby was discharged on 7th day. Grossly the mass presented white surface, firm consistency with small hemorrhages on cut surface. Microscopically the nodule, encircled by a fibrous pseudo-capsule, was mostly composed of gemistocytic astrocytes, occasionally binucleated, interspersed within fibrillary neuroglial tissue. Strands of fibrous tissue, in continuity with the pseudo-capsule, separated the glial tissue. No neuronal cells were seen. Necrosis, mitotic figures and vascular proliferations were absent. GFAP immunohistochemical stain confirmed the glial nature of the cells. Our diagnosis was one of "heterotopic glial tissue of nose" (nasal glioma). The absence of connection between the nodule and endocranial contents (CSF-filled spaces, leptomeningeal or dural tissue), excluded the diagnosis of encephalocele. In our case, the tissue was only of embryonic neuroectodermal derivation: on this basis the diagnosis of teratoma, which is classically composed of two or three embryonic layers could be excluded. The pathogenesis of nasal glioma is briefly discussed by authors.
一名6个月大的女婴因面部先天性畸形入住我院:左侧鼻区有一个皮下结节。X线检查显示鼻中隔有明显侧弯。手术中一个白色坚实的结节未完全切除;术后CT扫描排除了肿瘤与脑的任何连通。未见骨腔隙。临床上既无鼻漏也无脑膜炎。婴儿于第7天出院。大体上,肿块表面白色,质地坚实,切面有小出血点。显微镜下,结节被纤维性假包膜环绕,主要由肥胖型星形胶质细胞组成,偶尔双核,散在于纤维性神经胶质组织中。与假包膜连续的纤维组织束将神经胶质组织分隔开。未见神经元细胞。无坏死、有丝分裂象及血管增生。GFAP免疫组化染色证实了细胞的神经胶质性质。我们的诊断是“鼻异位神经胶质组织”(鼻胶质瘤)。结节与颅内内容物(充满脑脊液的间隙、软脑膜或硬脑膜组织)之间无连接,排除了脑膨出的诊断。在我们的病例中,该组织仅来源于胚胎神经外胚层:基于此,可以排除经典的由两三个胚胎层组成的畸胎瘤的诊断。作者简要讨论了鼻胶质瘤的发病机制。