Olfactory neuroepithelioma arising from the olfactory placode.
Olfactory neuroepithelioma arising from the olfactory placode.
复制标题
嗅神经上皮瘤起源于嗅基板。
作者：
S. Utsuki;N. Kawano;H. Oka;S. Shimizu;T. Sagiuchi;H. Saegusa;K. Fujii;S. Yagishita;H. Okabe
患者是一名 54 岁男性，6 年前就失去了嗅觉，并在入院前约 6 个月开始变得健忘。入院 MRI 显示一个巨大的多囊性肿瘤，Gd-DTPA 增强，从前颅窝延伸穿过蝶窦，进入鼻腔。组织病理学检查显示被结缔组织隔膜分开的小圆形细胞广泛增殖。尽管不存在基底膜，但肿瘤细胞偶尔形成管状结构。免疫染色显示，圆形肿瘤细胞的神经元特异性烯醇化酶、突触素和嗜铬粒蛋白 A 呈阳性，而形成小管的细胞的 AE 1 和 CAM 5.2 呈阳性。几乎所有肿瘤细胞的 Ber-EP4 呈阳性，管状结构周围的一些上皮样细胞的黄体生成素释放激素 (LH-RH) 也呈阳性。电子显微镜显示零星的细胞间连接、肿瘤细胞突起中的许多微管以及细胞质中的透明核和致密核囊泡。根据结果，该病例似乎是第一个有记录的神经上皮瘤，其中 Ber-EP4 和 LH-RH 阳性细胞源自嗅基板。
The patient was a 54-year-old man, who had lost his sense of smell 6 years previously and had started to become forgetful about 6 months prior to presenting at hospital. MRI admission showed a large multicystic tumor with Gd-DTPA enhancement extending from the anterior cranial fossa through the sphenoid sinus and into the nasal cavity. Histopathological examination revealed extensive proliferation of small round cells that were divided by connective tissue septae. The tumor cells occasionally formed tubular structures, although no basement membranes were present. On immunostaining, round tumor cells were positive for neuron-specific enolase, synaptophysin, and chromogranin A, while cells forming tubules were positive for AE 1 and CAM 5.2. Almost all of the tumor cells were positive for Ber-EP4, and some of the epithelioid cells surrounding the tubular structures were also positive for luteinizing hormone-releasing hormone (LH-RH). Electron microscopy demonstrated sporadic intercellular junctions, many microtubules in the tumor cell processes, and clear- and dense-cored vesicles in the cytoplasm. Based on the results, this case appears to be the first documented neuroepithelioma with Ber-EP4- and LH-RH-positive cells arising from the olfactory placode.