Haroon Saroona, Tariq Muhammad Usman, Memon Aisha, Fatima Saira, Hasan Sheema Habibul
Dr. Saroona Haroon, MBBS, FCPS (Histopathology). Consultant and Histopathology Dept. Chair, Department of Pathology and Laboratory Medicine, Prince Faisal Cancer Centre, King Fahad Specialist Hospital, Buraidah, Kingdom of Saudi Arabia.
Dr. Muhammad Usman Tariq, MBBS, FCPS (Histopathology). Instructor, Section of Histopathology, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan.
Pak J Med Sci. 2016 May-Jun;32(3):585-90. doi: 10.12669/pjms.323.9738.
To present the clinicopathological experience of Olfactory Neuroblastoma (ONB) with emphasis on histopathological and immunohistochemical features.
A descriptive cross-sectional study was done on 36 cases of ONB, selected by non-probability purposive sampling. Theses cases of ONB were retrieved and reviewed from surgical pathology database of Aga Khan University Hospital reported between January 1993 and March 2015.
Tumor size and age of presentation was wide in range without any distinct bimodal distribution. Nasal cavity was most common site along with involvement of paranasal sinuses. More than 50% cases had Kadish stage A. Microscopically, most cases were Grade-1 and majority showed partial or complete lobular architecture. Neurofibrillary matrix was observed in 2/3(rd) of cases. Among immunohistochemical markers, Neuron Specific Enolase was most frequently expressed. Unusual positive expression of Cytokeratin AE1/AE3 and Cytokeratin CAM5.2 was also seen focally in few cases.
The ONB has great variability of histological and clinical presentation, and immunohistochemical markers are useful to differentiate from more common small round blue cell tumours of nasal cavity.
介绍嗅神经母细胞瘤（ONB）的临床病理经验，重点关注组织病理学和免疫组化特征。
采用非概率目的抽样法，对36例ONB进行描述性横断面研究。这些ONB病例从阿迦汗大学医院1993年1月至2015年3月报告的外科病理数据库中检索并回顾。
肿瘤大小和发病年龄范围广泛，无明显的双峰分布。鼻腔是最常见的部位，同时鼻窦也会受累。超过50%的病例为卡迪什A期。显微镜下，大多数病例为1级，多数表现为部分或完全小叶结构。2/3的病例观察到神经原纤维基质。在免疫组化标志物中，神经元特异性烯醇化酶表达最频繁。少数病例还局部观察到细胞角蛋白AE1/AE3和细胞角蛋白CAM5.2的异常阳性表达。
ONB在组织学和临床表现上具有很大的变异性，免疫组化标志物有助于与鼻腔更常见的小圆形蓝细胞肿瘤相鉴别。
