Carney M E, O'Reilly R C, Sholevar B, Buiakova O I, Lowry L D, Keane W M, Margolis F L, Rothstein J L
Department of Otolaryngology-Head and Neck Surgery, Jefferson Medical College, Jefferson Cancer Institute, Philadelphia, PA 19107, USA.
J Neurooncol. 1995 Oct;26(1):35-43. doi: 10.1007/BF01054767.
Olfactory neuroblastoma (ONB) is a rare neuronal malignancy of the olfactory mucosal. Markers used in the diagnosis of ONB do not distinguish ONB from other neuronal tumors or tumors with neuroendocrine features thus making the diagnosis of ONB difficult. Using a modified RT-PCR technique, we show that the human homologue of the Drosophila achaete-scute gene HASH1 is expressed in 6 primary and one metastatic ONB specimens, whereas Olfactory Marker Protein (OMP) is not. Previous studies have shown that HASH1 is expressed in immature olfactory neurons and is required for their development. OMP, whose function is unknown, is expressed exclusively in mature olfactory neurons. Together, these data suggest that ONB is derived from immature olfactory neurons of neuroectodermal origin. Analysis of RNA expression in primary tumor specimens and in an established cell line make this an ideal system to study olfactory growth and differentiation. Furthermore, these studies represent the first molecular genetic analysis of this rare and unusual neuronal tumor.
嗅神经母细胞瘤(ONB)是一种罕见的嗅黏膜神经元恶性肿瘤。用于ONB诊断的标志物无法将ONB与其他神经元肿瘤或具有神经内分泌特征的肿瘤区分开来,因此ONB的诊断较为困难。我们采用改良的逆转录聚合酶链反应(RT-PCR)技术,发现果蝇achaete-scute基因的人类同源物HASH1在6例原发性和1例转移性ONB标本中表达,而嗅觉标记蛋白(OMP)则不表达。先前的研究表明,HASH1在未成熟的嗅觉神经元中表达,并且是其发育所必需的。功能未知的OMP仅在成熟的嗅觉神经元中表达。这些数据共同表明,ONB起源于神经外胚层来源的未成熟嗅觉神经元。对原发性肿瘤标本和已建立的细胞系中的RNA表达进行分析,使其成为研究嗅觉生长和分化的理想系统。此外,这些研究代表了对这种罕见且特殊的神经元肿瘤的首次分子遗传学分析。