垂体多激素PIT1谱系神经内分泌肿瘤临床病理学观察  

Plurihormonal PIT1-lineage pituitary neuroendocrine tumors:a clinicopathological study

作　　者：段泽君[1] 冯景 赵和千 王海丹[3] 桂秋萍 张绪妃 马忠 胡泽娟 向磊 齐雪岭[1] Duan Zejun;Feng Jing;Zhao Heqian;Wang Haidan;Gui Qiuping;Zhang Xufei;Ma Zhong;Hu Zejuan;Xiang Lei;Qi Xueling(Department of Pathology,Sanbo Brain Hospital,Capital Medical University,Beijing 100093,China;Department of Neurosurgery,Sanbo Brain Hospital,Capital Medical University,Beijing 100093,China;Department of Neurology,Sanbo Brain Hospital,Capital Medical University,Beijing 100093,China;Department of Radiology,Sanbo Brain Hospital,Capital Medical University,Beijing 100093,China)

机构地区：[1]首都医科大学三博脑科医院病理科,北京100093 [2]首都医科大学三博脑科医院神经外科三病区,北京100093 [3]首都医科大学三博脑科医院神经内科,北京100093 [4]首都医科大学三博脑科医院影像科,北京100093

出　　处：《中华病理学杂志》2023年第10期1017-1024,共8页Chinese Journal of Pathology

摘　　要：目的:探讨垂体多激素PIT1谱系神经内分泌肿瘤的临床病理学特点。方法:收集首都医科大学三博脑科医院2018年1月至2022年4月病理数据库中诊断为多激素PIT1谱系肿瘤的垂体神经内分泌肿瘤病例,整理相关临床及影像学资料,进行HE、免疫组织化学及特殊染色。结果:48例多激素PIT1谱系肿瘤纳入研究,成熟性多激素PIT1谱系肿瘤13例,不成熟性PIT1谱系肿瘤35例,研究显示两组肿瘤的临床及病理学特征均存在明显差异。临床特征:(1)成熟性多激素PIT1谱系肿瘤几乎均有垂体激素分泌增多的临床症状,而不成熟性PIT1谱系肿瘤少见,但有62.9%(22/35)的患者存在血清垂体激素低水平升高;(2)不成熟性PIT1谱系肿瘤患者偏年轻化,肿瘤体积更大,影像学易见侵袭周围组织。病理学特征:(1)成熟性多激素PIT1谱系肿瘤由大的嗜酸性细胞组成,生长激素表达比例高,而不成熟性PIT1谱系肿瘤由嫌色性细胞组成,催乳素表达比例更高;(2)成熟性多激素PIT1谱系肿瘤表现为一致性、弥漫性胞质广谱细胞角蛋白阳性,而不成熟性PIT1谱系肿瘤细胞角蛋白表达则具多样性;(3)不成熟性PIT1谱系肿瘤增殖明显活跃;(4)25.0%(12/48)多激素PIT1谱系肿瘤存在性激素的异常表达。使用Kaplan-Meier法进行肿瘤无进展时间分析,两组肿瘤间差异无统计学意义( P=0.648)。 结论:多激素PIT1谱系肿瘤属于少见的PIT1谱系垂体神经内分泌肿瘤类型,以不成熟性PIT1谱系肿瘤多见。依据垂体激素分泌增多的临床症状、嗜酸性肿瘤细胞、高比例生长激素表达、细胞角蛋白弥漫性胞质阳性和低增殖活性,可以协助成熟性多激素PIT1谱系肿瘤与不成熟性PIT1谱系肿瘤的鉴别。不成熟性PIT1谱系肿瘤临床病理学比较复杂。ObjectiveTo investigate the clinicopathological characteristics of plurihormonal PIT1-lineage pituitary neuroendocrine tumors.MethodsForty-eight plurihormonal PIT1-lineage tumors were collected between January 2018 and April 2022 from the pathological database of Sanbo Brain Hospital,Capital Medical University.The related clinical and imaging data were retrieved.H&E,immunohistochemical and special stains were performed.ResultsOut of the 48 plurihormonal PIT1-lineage tumors included,13 cases were mature PIT1-lineage tumors and 35 cases were immature PIT1-lineage tumors.There were some obvious clinicopathological differences between the two groups.Clinically,the mature plurihormonal PIT1-lineage tumor mostly had endocrine symptoms due to increased hormone production,while a small number of immature PIT1-lineage tumors had endocrine symptoms accompanied by low-level increased serum pituitary hormone;patients with the immature PIT1-lineage tumors were younger than the mature PIT1-lineage tumors;the immature PIT1-lineage tumors were larger in size and more likely invasive in imaging.Histopathologically,the mature PIT1-lineage tumors were composed of large eosinophilic cells with high proportion of growth hormone expression,while the immature PIT1-lineage tumors consisted of chromophobe cells with a relatively higher expression of prolactin;the mature PIT1-lineage tumors had consistently diffuse cytoplasmic positive staining for keratin,while the immature PIT1-lineage tumors had various expression for keratin;the immature PIT1-lineage tumors showed more mitotic figures and higher Ki-67 proliferation index;in addition,25.0%(12/48)of PIT1-positive plurihormonal tumors showed abnormal positive staining for gonadotropin hormones.There was no significant difference in the progression-free survival between the two groups(P=0.648)by Kaplan-Meier analysis.ConclusionsPlurihormonal PIT1-lineage tumor belongs to a rare type of PIT1-lineage pituitary neuroendocrine tumors,most of which are of immature lineage.Clinically increased

关 键 词：垂体肿瘤 神经内分泌瘤 垂体激素类 多激素PIT1谱系肿瘤 

分 类 号：R736.4[医药卫生—肿瘤]