CAJ | 학술논문

目的观察嗅神经母细胞瘤(ONB)的特殊病理结构,探求其病理意义及其与预后的关系.方法对40例ONB进行病理形态及免疫组织化学SP法染色观察,结合随访资料对观察结果进行统计分析.结果 ONB在光镜下典型的病理结构为肿瘤细胞分叶状排列、神经原纤维背景、血管襻间质及真、假菊形团;特殊的病理结构包括黏液腺上皮、鳞状上皮巢,所占比例分别为45.0%(18/40)、15.0%(6/40);3例可见到嗅上皮内原位肿瘤;2例可见肿瘤突向黏膜表面呈外生性乳头状生长.根据Hyams分级标准对ONB进行分级,Log-rank生存分析显示Hyams分级与患者的生存率无显著相关性(P>0.05),而肿瘤组织内出现坏死与患者生存率呈负相关(P=0.016),出现黏液腺上皮结构与患者生存率呈正相关(P=0.011).结论 ONB的特殊病理结构包括上皮性结构、嗅上皮内原位肿瘤、肿瘤突向黏膜表面呈外生性乳头状生长.ONB的典型及特殊病理结构提示ONB可能起源于嗅上皮的未分化基细胞,后者具有双向分化的潜能.ONB中出现坏死和黏液腺上皮结构与患者的预后相关.
목적 관찰후신경모세포류(ONB)적특수병리결구,탐구기병리의의급기여예후적관계.방법 대40례ONB진행병리형태급면역조직화학SP법염색관찰,결합수방자료대관찰결과 진행통계분석.결과 ONB재광경하전형적병리결구위종류세포분협상배렬、신경원섬유배경、혈관반간질급진、가국형단;특수적병리결구포괄점액선상피、린상상피소,소점비례분별위45.0%(18/40)、15.0%(6/40);3례가견도후상피내원위종류;2례가견종류돌향점막표면정외생성유두상생장.근거Hyams분급표준대ONB진행분급,Log-rank생존분석현시Hyams분급여환자적생존솔무현저상관성(P>0.05),이종류조직내출현배사여환자생존솔정부상관(P=0.016),출현점액선상피결구여환자생존솔정정상관(P=0.011).결론 ONB적특수병리결구포괄상피성결구、후상피내원위종류、종류돌향점막표면정외생성유두상생장.ONB적전형급특수병리결구제시ONB가능기원우후상피적미분화기세포,후자구유쌍향분화적잠능.ONB중출현배사화점액선상피결구여환자적예후상관.
Objective To investigate unusual pathological features of olfactory neuroblastoma (ONB) and its correlation with the clinical prognosis. Methods Totally 40 cases of ONB were studied using histology and immunohistochemistry techniques. All the cases of ONB were graded according to Hyams Grading system. Results ONB consisted of small round tumor cells growing in nests or lohules separated by fibrovascular septa. Characteristically,there were neurofibrillary intercellular matrices and Homer-Wright or Flexner-Wintersteiner rosette formation. The unusual structures including epithelial components such as mucous or squamons cell nests which were found in 45.0% (18/40), and 15.0% (6/40) respectively. In addition, 3 cases showed an in-situ form with invasion of tumor into olfactory epithelium, and there was exogenous papillary proliferation seen in 2 cases. Log-rank survival analysis demonstrated that Hyams Grading had no statistical correlation with the prognosis. The presence of necrosis was correlated with a poor prognosis (P=0.016) while the presence of mucous cells was correlated with a good prognosis (P=0.011). Conclusions Unusual pathological structures including epithelial structures, in-situ invasion of tumor tissue into the involving olfactory epithelium and exogenous papillary proliferation can be found in ONB, suggesting that ONB may originate from the undifferentiated basal cells of olfactory epithelium,through bipotential differentiation. The presence of tumor necrosis in ONB is a poor prognostic indicator while the presence of mucous cells suggests a good prognosis.