胸椎良性脊索细胞瘤临床病理及文献复习

Benign Notochordal Cell Tumor of Thoracic Vertebrae:Clinical Pathologyand Review of Literatures

【目的】探讨良性脊索细胞瘤(BNCT)的临床病理特征、诊断及鉴别诊断。【方法】回顾本院1例胸椎内良性脊索细胞瘤患者的临床及影像学资料,分析BNCT的组织形态、免疫组化表达特征并复习相关文献。【结果】本院就诊患者男性,59岁,无明显诱因背痛1年。MRI显示胸11椎体内T1WI低信号和T2WI高信号影,边缘清楚,无软组织肿块。因穿刺结果提示可能为脊索瘤,遂行胸11椎体切除及胸椎重建术。病理取材肉眼见破碎骨质,未见明显肿物。镜下骨小梁间见多灶性、成片分布的"脂肪样"细胞,呈马赛克样镶嵌排列,胞质空亮或嗜酸(HE染色),细胞核圆形或卵圆形,被挤压于细胞边缘,几乎无异型性或少量细胞仅见轻度异型性,未见核分裂象。肿瘤不形成分叶状结构,亦未见黏液基质。免疫组化示瘤细胞CK、Vimentin、EMA、S-100均为阳性。【结论】BNCT是一种少见的脊索源性良性肿瘤,与脊索瘤的临床处理方式不同。因与脊索瘤免疫表型有重叠,易造成误诊,需要充分认识,避免过度治疗。

[ Objective ] This study was designed to investigate the clinicopathological features and diagnosis of benign notochordal cell tumor （BNCT） ,as well as the differential diagnosis with chordoma. [Methods] Clinical and radiological data form one case of BNCT were collected; the morphology and immunohistoehemieal pattern were analyzed. Related literatures were reviewed. [ Results ] One 59-year-old man was admitted to The Third Affiliated Hospital, Sun Yat-sen University for 1-year back pain without significant incentives. MRI showed an occupied lesion demonstrated hyperintensity on T2WI and hypointense on T1WI within the eleventh thoracic vertebrae without soft tissue mass. Then the patient underwent corpectomy and lumbar reconstruction.Grossly, a bunch of broken bone was inspected but no visualized mass was found. Microscopically, multifocal lesions were distributed between the trabecular, the neoplasm composed predominantly of solid sheets of adipocyte-likecells, it arranged in a mosaic pattern with distinct membranes, vacuolated or eosinophilic cytoplasm, with round or oval nuclei eccentrically, almost without atypia or only slightly atypia, no mitotic was found. The tumor had no lobulated structure and myxoid matrix. Immunohistoehemistry showed positive of CK, Vimentin, EMA, and S-100. [Conclusion] BNCT is a rare benign tumor derived from notochord with different treatment compared to chordoma. The overlap of immune phenotype between them may lead to misdiagnosis, fully aware of it may avoid over-treatment. Keywords ： benign notochordal cell tumors ; chordoma; clinical pathology ; immunohistoehemistry