Sinonasal Tumors With Neuroepithelial Differentiation (Olfactory Carcinoma)

病理 神经上皮细胞 感觉神经母细胞瘤 组织发生 生物 鼻腔 解剖 医学 免疫组织化学 干细胞 遗传学 神经干细胞
作者
Lisa Rooper,Justin A. Bishop,William C. Faquin,Robert D. Foss,Gary L. Gallia,Vickie Y. Jo,James S. Lewis,Michiya Nishino,Edward B. Stelow,Lester D.�R. Thompson,Bruce M. Wenig,William H. Westra
出处
期刊:The American Journal of Surgical Pathology [Lippincott Williams & Wilkins]
卷期号:46 (8): 1025-1035 被引量:28
标识
DOI:10.1097/pas.0000000000001908
摘要

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.
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