摘要
十二指肠复合神经节细胞瘤/神经瘤-神经内分泌肿瘤(composite gangliocytoma/neuroma and neuroendocrine tumor,CoGNET)是一种较罕见的肿瘤.CoGNET先前被称为节细胞性副神经节瘤(gangliocytic paraganglioma,GP),在2022年世界卫生组织神经内分泌肿瘤分类中,其被重新命名.本文报告1例65岁CoGNET男性患者,因反酸、烧心伴便血就诊.术后病理显示肿物由梭形细胞、神经节细胞和上皮样细胞3种细胞成分组成.3种细胞成分具有不同的免疫组织化学染色模式:梭形细胞示神经丝蛋白(neurofilament protein,NFP)(+),中枢神经特异蛋白(central nervous system specific protein,S-100)(+),与性别决定区域Y相关的高迁移率组盒蛋白10(sex deciding region y-box[SRY]-related high motility[HMG]-box 10,SOX10)(+);神经节细胞示NFP(+),S-100(+),SOX10(-);上皮样细胞示广谱细胞角蛋白(pan cytokeratin,AE1/AE3)(+),细胞黏附分子5.2(cell adhesion molecules 5.2,CAM5.2)(+),突触素(synaptophysin,Syn)(+),嗜铬素A(chromogranin A,CgA)(+),孕激素受体(progesterone receptor,PR)(+),GATA结合蛋白3(GATA binding protein 3,GATA-3)(-),胶质纤维酸性蛋白(glial fibrillary acidic protein,GFAP)(-),分化抗原簇117(cluster of differentiation 117,CD117)(弱+).病理诊断为CoGNET,神经内分泌肿瘤成分为高分化神经内分泌瘤.患者术后未行进一步治疗,术后随访19个月,无复发及转移.
Abstract
Duodenal composite gangliocytoma/neuroma and neuroendocrine tumor(CoGNET)is a rare tumor.Previously referred to as gangliocytic paraganglioma(GP),it was renamed in the 2022 World Health Organization Classification of neuroendocrine tumors.We report a case of 65-year-old male CoGNET patient who presented with acid reflux,heartburn,and bloody stools.Postoperative pathology revealed a tumor composed of 3 cell types:spindle cells,ganglion cells,and epithelioid cells,each with distinct immunohistochemical staining patterns.Spindle cells tested positive for neurofilament protein(NFP),central nervous system specific protein(S-100),and sex deciding region y-box[SRY]-related high motility[HMG]-box 10(SOX10);ganglion cells tested positive for NFP and S-100,but negative for SOX10;epithelioid cells tested positive for pan cytokeratin(AE1/AE3),cell adhesion molecules 5.2(CAM5.2),synaptophysin(Syn),chromogranin A(CgA),progesterone receptor(PR),and showed weak positivity for cluster of differentiation 117(CD117),while negative for GATA binding protein 3(GATA-3)and glial fibrillary acidic protein(GFAP).The final pathological diagnosis was CoGNET,with the neuroendocrine tumor component classified as a well-differentiated neuroendocrine tumor G1.The patient received no additional treatment post-surgery and was followed up for 19 months without recurrence or metastasis.
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