CAJ | 학술논문

目的：报道2例2013版WHO软组织和骨肿瘤分类新增加的罕见病种：良性脊索细胞瘤（BNCT），探讨其临床、影像和病理学特征及病理鉴别诊断。方法收集2例良性脊索细胞瘤的临床及影像学资料，通过光镜观察及免疫组织化学EnVision法分析其临床、影像学、病理学特征、免疫表型及病理鉴别诊断，并复习相关文献。结果例1为53岁的男性患者，例2为61岁的女性患者，影像学均为第5颈椎椎体信号异常，病变局限于骨内，无软组织肿块。镜下均为骨小梁间隙内片状分布的空泡状“脂肪样”细胞，边界清楚，胞质透明，细胞核中位或偏位，无异型性，未见核分裂象；细胞外无黏液样基质形成，缺乏分叶状结构；病变周围骨小梁常有硬化。结论良性脊索细胞瘤的好发部位及免疫表型与脊索瘤相似，但两者影像学及病理形态表现均不相同，并且有不同的治疗原则与预后，两者的鉴别有赖于影像、临床及病理的密切配合。
목적：보도2례2013판WHO연조직화골종류분류신증가적한견병충：량성척색세포류（BNCT），탐토기림상、영상화병이학특정급병리감별진단。방법수집2례량성척색세포류적림상급영상학자료，통과광경관찰급면역조직화학EnVision법분석기림상、영상학、병이학특정、면역표형급병리감별진단，병복습상관문헌。결과례1위53세적남성환자，례2위61세적녀성환자，영상학균위제5경추추체신호이상，병변국한우골내，무연조직종괴。경하균위골소량간극내편상분포적공포상“지방양”세포，변계청초，포질투명，세포핵중위혹편위，무이형성，미견핵분렬상；세포외무점액양기질형성，결핍분협상결구；병변주위골소량상유경화。결론량성척색세포류적호발부위급면역표형여척색류상사，단량자영상학급병리형태표현균불상동，병차유불동적치료원칙여예후，량자적감별유뢰우영상、림상급병리적밀절배합。
Objective To report 2 rare cases of benign notochordal cell tumor ( BNCT) , according to WHO classification of tumors of soft tissue and bone (4th edition).Their radiologic and clincopathologic features and differential diagnosis were investigated.Methods Two cases of BNCT were studied by retrospective review of the clinical , radiologic, pathologic and immunophenotypical findings.Related literatures were reviewed at the same time.Results Case 1 was a 53-year-old man, and case 2 was a 61-year-old woman.Radiographically , both patients presented with abnormal imaging findings in the fifth cervical vertebral body with the lesions located within the bone but without extra osseous mass .Histopathologically , the lesions lacked lobular architecture and extracellular myxoid matrix.The tumor cells were vacuolated and had centrally or peripherally placed round or oval nuclei with small nucleoli , mimicking mature adipocytes.No cytological atypia or mitotic figures were seen.The affected bone trabeculae were sclerotic and islands of bone marrow were often entrapped within the tumor.Conclusions Although sharing similar anatomic distribution and immunophenotype to those of chordoma , BNCT has distinct radiologic and pathologic features and different treatment and prognosis.The differential diagnosis between BNCT and chordoma requires detailed clinical , radiologic and histopathologic evaluations.