嗅神经母细胞瘤临床病理分析

A clinicopathological study of olfactory neuroblastoma

目的探讨嗅神经母细胞瘤患者的临床病理特征、诊断与鉴别诊断，提高病理诊断的准确性。方法收集13例嗅神经母细胞瘤的临床病理学资料，结合文献对其临床表现、病理形态特点及鉴别诊断进行分析。结果13例患者中，男性10例，女性3例，年龄17～59岁。镜下：肿瘤组织排列成巢状、小叶状，肿瘤细胞大小一致，胞质稀少。细胞核小，圆形或卵圆形，核染色质呈“椒盐样”。部分可见Homer—Wright假菊形团及Flexner—Wintersteiner真菊形团。免疫组化：大部分病例肿瘤细胞表达Syn、CD56、CgA、S-100，Ki-67增殖指数15％～80％。6例患者有随访资料，随访时间4个月～6年，局部复发5例，其中2例伴转移。结论嗅神经母细胞瘤是发生于鼻腔的一种少见的恶性肿瘤，侵袭性强，组织形态与鼻腔其他肿瘤有重叠，尚无特异的分子标记物，寻找特征性组织学表现，辅以免疫组化及分子检测排除其他小圆细胞恶性肿瘤有助于确诊。

Objective To analyze the clinical and pathological features, diagnosis and differential diagnosis of olfac- tory neuroblastoma （ONB） , and enhance the accuracy of pathological diagnosis of the disease. Methods The clinical data, histological observation and immunohistochemical staining results were collected from 13 of olfactory neuroblastoma patients before analysis. Meanwhile, related literatures were reviewed. Results The patients （ 10 men and 3 women） aged from 17 to 59 years old. Histologically, the tumor was composed of cells with the same size and scant cytoplasm ar-ranged in lobules and nests. The tumor cells were poorly delineated and had small round nuclei with salt - and pepper chromatin. Homer- Wright and Flexner- Wintersteiner rosettes were found in some samples. Immunohistochemieally, the cells were typically positive for Syn, CD56, CgA, S - 100, and Ki -67 -labeling indices ranged from 15% to 80%. Also, 6 patients were follow - up visited from 4 months to 6 years, where 5 patients reported local recurrence and 2 presented metastasis. Conclusions Olfactory neuroblastoma is a rare malignant tumor with strong invasiveness. Its definite diagnosis comes from unique morphology, immunohistochemical expression and additional gene analysis.