嗅神经母细胞瘤：1例的超微结构、免疫组织化学及生化研究

Esthesioneuroblastoma: ultrastructural, immunohistological and biochemical investigation of one case.

作者信息

Vollrath M, Altmannsberger M, Hunneman D H, Goebel H H, Osborn M

出版信息

Arch Otorhinolaryngol. 1984;239(2):133-44. doi: 10.1007/BF00463554.

Abstract

A case of esthesioneuroblastoma, the pathological diagnosis of which almost always causes great difficulties, was investigated ultrastructurally, biochemically, and immunohistologically, using antibodies against the five known types of intermediate filaments [keratin, vimentin, desmin, glial fibrillary acidic protein (GFAP) and neurofilaments]. The tumour cells did not react with antibodies against any of the five intermediate filament proteins. Ultrastructural investigations showed dense cored secretory granules in the cytoplasm and cell processes. Thus, immunohistology offers by "exclusion" a differential diagnosis to avoid often misdiagnosed tumours (undifferentiated carcinomas, embryonal rhabdomyosarcomas, and malignant lymphomas), since carcinomas react with antikeratin, embryonal rhabdomyosarcomas with antibodies to desmin and malignant lymphomas show immunofluorescence with antibodies to vimentin. The biological behaviour (age distribution, tendency to metastasize), the normal values of biochemical parameters, homovanillic acid and vanilmandelic acid (HVA, VMA), and the absence of neurofilaments distinguish this type of tumour from the peripheral sympathetic neuroblastoma.

摘要

对一例嗅神经母细胞瘤进行了超微结构、生化及免疫组织化学研究，该肿瘤的病理诊断几乎总是存在很大困难，研究中使用了针对五种已知类型中间丝（角蛋白、波形蛋白、结蛋白、胶质纤维酸性蛋白（GFAP）和神经丝）的抗体。肿瘤细胞与针对这五种中间丝蛋白中任何一种的抗体均无反应。超微结构研究显示，细胞质和细胞突起中有密集核心的分泌颗粒。因此，免疫组织化学通过“排除法”提供鉴别诊断，以避免经常被误诊的肿瘤（未分化癌、胚胎性横纹肌肉瘤和恶性淋巴瘤），因为癌与抗角蛋白抗体反应，胚胎性横纹肌肉瘤与抗结蛋白抗体反应，恶性淋巴瘤与抗波形蛋白抗体显示免疫荧光。该肿瘤的生物学行为（年龄分布、转移倾向）、生化参数同香草酸和香草扁桃酸（HVA、VMA）的正常数值以及神经丝的缺乏，将这种肿瘤与周围交感神经母细胞瘤区分开来。