良性脊索细胞瘤3例并文献复习

Benign notochordal cell tumor: report of 3 cases and review of literature

目的探讨良性脊索细胞瘤(benign notochordal cell tumor,BNCT)的临床病理学特征、诊断和鉴别诊断。方法回顾性分析3例BNCT临床病理学和免疫表型特征,并复习相关文献。结果 3例BNCT中女性2例,男性1例,年龄分别为27、59和29岁。眼观:3例肿瘤均为胶冻状碎组织,灰白、灰黄色,质软、脆。镜检:肿瘤由胞质透亮的脂肪样细胞和胞质淡染细胞构成,两种细胞呈巢状或片状排列。肿瘤细胞形态温和,未见核分裂象。肿瘤组织中未见分叶状结构、细胞外黏液样基质和坏死。免疫表型:肿瘤细胞均高表达CKpan、CK7、CK18、EMA、vimentin和S-100。结论 BNCT是一种罕见的良性脊索源性肿瘤,预后较好。BNCT需与脊索瘤、转移癌和脂肪组织鉴别。

Purpose To investigate the clinicopathological features, diagnosis and differential diagnosis of benign notochordal cell tumor （BNCT）. Methods Three cases of BNCT were studied for elinieopathologieal and immunohistochemical features, the available literature on this subject was reviewed as well. Results The 3 patients with BNCT, including 2 females and 1 male, were 27, 59, and 29 years old, respectively. On macroscopic examination, the samples of 3 excised tumors were multiple fragments and soft, friable, gelatinous, and yellowish-white in color. Histologically, the tumors were mainly composed of nests and sheets of adipocyte-like cells with clear cytoplasm and cells with eosinophilic cytoplasm. The tumor cells were bland and the mitotic figures were absent. The expansive lobular structure, extraeellular myxoid matrix and necrosis were completely absent in the current 3 tumors. Immunohisto-chemistry revealed that the tumor cells were strongly positive for CKpan, CKT, CK18, EMA, vimentin, and S-100. Conclusions BNCT is a rare benign tumor of notochord origin with good prognosis and it should be differentiated from chordoma, metastatic carcinoma and mature adipose tissue.