Olfactory carcinoma is one of many names applied to sinonasal malignancies with histologic similarity to olfactory neuroblastoma (ONB) but cytokeratin expression or gland formation. It is unclear whether these neuroepithelial tumors represent a unified category and if they are separate from ONB and currently-recognized sinonasal carcinomas. This study aims to explore their clinicopathologic characteristics based on a large collective experience. A total of 53 sinonasal tumors with neuroepithelial differentiation were identified affecting 41 men and 12 women, median age 47 years (range: 12 to 82 y). The vast majority arose in the superior nasal cavity and presented at the high Kadish-Morita stage. Frequent histologic findings included (1) lobulated and solid growth, (2) rosettes and/or neurofibrillary stroma, (3) high-grade cytology, (4) complex, often ciliated glands, (5) nonfocal pancytokeratin expression, (6) neuroendocrine pos+itivity, and (7) variable S100-positive sustentacular cells. Twelve patients with available follow-up (48%) developed progressive disease at a median 8 months (range: 0 to 114 mo to progression), and 7 (28%) died of disease. Despite disparate historical terminology, neuroepithelial differentiation is a recurrent and recognizable histologic pattern that is associated with aggressive behavior in sinonasal tumors. While tumors with this phenotype may originate from olfactory mucosa, well-developed epithelial features warrant separation from conventional ONB and neural elements distinguish them from most sinonasal carcinomas. Although their full histogenesis remains uncertain and some heterogeneity may exist, we propose that this pattern is sufficiently distinctive to merit separate recognition as olfactory carcinoma. Use of consistent nomenclature may facilitate greater recognition of tumors with this phenotype and understanding of their pathogenesis and classification.

嗅癌是鼻窦恶性肿瘤的众多名称之一，其组织学与嗅神经母细胞瘤 (ONB) 相似，但有细胞角蛋白表达或腺体形成。目前尚不清楚这些神经上皮肿瘤是否代表一个统一的类别，以及它们是否与 ONB 和目前公认的鼻窦癌分开。本研究旨在根据大量集体经验探讨其临床病理特征。总共确定了 53 个具有神经上皮分化的鼻窦肿瘤，影响 41 名男性和 12 名女性，中位年龄 47 岁（范围：12 至 82 岁）。绝大多数发生于上鼻腔，并出现在卡迪什-森田阶段的高位。常见的组织学发现包括（1）分叶状和实性生长，（2）玫瑰花结和/或神经原纤维基质，（3）高级细胞学，（4）复杂的，通常有纤毛的腺体，（5）非局灶性全细胞角蛋白表达，（6）神经内分泌pos+itivity，和（7）可变的S100阳性支持细胞。12 名进行随访的患者 (48%) 在中位 8 个月时出现疾病进展（范围：进展 0 至 114 个月），7 名患者 (28%) 死于疾病。尽管历史术语不同，但神经上皮分化是一种反复出现且可识别的组织学模式，与鼻窦肿瘤的侵袭行为相关。虽然具有这种表型的肿瘤可能起源于嗅粘膜，但发达的上皮特征保证了与传统 ONB 的分离，并且神经元件将它们与大多数鼻窦癌区分开来。尽管它们的完整组织发生仍不确定，并且可能存在一些异质性，但我们认为这种模式足够独特，值得单独识别为嗅癌。使用一致的命名法可能有助于更好地识别具有这种表型的肿瘤并了解其发病机制和分类。