2例膀胱纯上皮性神经内分泌肿瘤临床病理特征差异及文献复习  

Pure epithelial neuroendocrine neoplasms of the bladder:clinicopathological characteristics of 2 cases and litera⁃ture review

作　　者：周恒花 林斓 朱桂香 刘敏 黄文涛 ZHOU Henghua;LIN Lan;ZHU Guixiang;LIU Min;HUANG Wentao(Department of Pathology,the Affiliated Tongren Hospital of Shanghai Jiaotong Univer-sity School of Medicine,Shanghai 200336,China;Department of Urology Surgery,the Affiliated Tongren Hospital of Shanghai Jiaotong Univer-sity School of Medicine,Shanghai 200336,China)

机构地区：[1]上海交通大学医学院附属同仁医院病理科,上海200336 [2]上海交通大学医学院附属同仁医院泌尿外科,上海200336

出　　处：《诊断学理论与实践》2024年第6期602-611,共10页Journal of Diagnostics Concepts & Practice

摘　　要：目的:探讨膀胱纯上皮性神经内分泌肿瘤(neuroendocrine neoplasms,NEN)的临床病理学特征和预后。方法:回顾性分析2018年至2024年间收治的2例膀胱纯上皮性NEN患者的临床资料、组织病理学形态以及免疫组织化学,并进行鉴别诊断和详细地文献复习。结果:本组2例均为女性,年龄分别为85岁(例1)和84岁(例2),分别因下肢水肿和血尿就诊。B超及MRI检查示,例1膀胱三角区见一息肉状低回声,MRI增强无强化,考虑良性病变可能;例2膀胱前壁内见一结节状低回声,MRI增强呈明显强化,考虑恶性肿瘤可能,副神经节瘤不除外。巨检,2例分别为息肉状组织及结节状组织各一枚,最大径0.6~3.0 cm,例1表面光滑,例2切面呈灰黄色、质中。镜下,2例肿瘤均呈典型NEN的组织病理学形态,表面均被覆膀胱黏膜。例1肿瘤完全位于黏膜固有层内,例2肿瘤位于固有肌层内呈浸润性生长。例1诊断为分化好的神经内分泌肿瘤(well-differentiated neuroendocrine tumors,WD-NET),例2为大细胞神经内分泌癌(large cell neuroendocrine carcinoma,LCNEC)。2例肿瘤细胞均弥漫阳性表达广谱细胞角蛋白及神经内分泌标志物(2/2),P53均呈野生型表达(2/2),例1肿瘤Ki-67增殖指数为3%~5%,局部表达PSAP,例2肿瘤Ki-67增殖指数达60%~70%,少量表达GATA-3。2例均未行特殊治疗,例1随访76个月,无瘤生存;例2术后3个月复发伴肺转移死亡。文献复习提示,膀胱纯上皮性NEN均具有典型NEN肿瘤的病理组织学形态及免疫表型,WD-NET多呈惰性经过,而LCNEC多具有高度侵袭性,易复发转移,与本文报道的2例病例一致。结论:膀胱纯上皮性NEN非常少见,明确诊断主要依据组织病理学检查和免疫组织化学染色,WD-NET完整切除后预后良好,而LCNEC具有高度侵袭性,预后不良。Objective To investigate the clinicopathological features and prognosis of pure epithelial neuroendo-crine neoplasms(NEN)of the bladder.Methods A retrospective analysis was conducted on the clinical data,histopatho-logical morphology,and immunohistochemistry of 2 patients with pure epithelial NEN of the bladder admitted between 2018 and 2024.A differential diagnosis was performed,and a detailed literature review was conducted.Results Both pa-tients were females,aged 85(Case 1)and 84(Case 2),presenting with lower limb edema and hematuria,respectively.B-scan ultrasound and MRI examinations showed a polypoid hypoechoic lesion in the trigone of the bladder in Case 1,with no enhancement on MRI,suggesting a benign lesion.Case 2 showed a nodular hypoechoic lesion in the anterior bladder wall with significant enhancement on MRI,suggesting a malignant tumor,with paraganglioma not excluded.Gross examination revealed that the two cases had one polypoid tissue and one nodular tissue,respectively,with maximum diameters ranging from 0.6 cm to 3.0 cm.Case 1 had a smooth surface,while Case 2 had a grayish-yellow cross section with medium hardness.Microscopically,both tumors showed typical NEN histopathological features,with the surface covered by bladder mucosa.Case 1 was confined to the lamina propria,while Case 2 showed invasive growth into the muscularis propria.Case 1 was di-agnosed as well-differentiated neuroendocrine tumor(WD-NET)and Case 2 as large cell neuroendocrine carcinoma(LCNEC).Immunohistochemically,both tumor cells diffusely expressed broad-spectrum cytokeratin and neuroendocrine markers(2/2),with wild-type expression of P53(2/2).The Ki67 proliferation index was 3%-5%in Case 1,with focal PSAP expression,and 60%-70%in Case 2,with minimal GATA-3 expression.Neither patient received special treatment.Case 1 was followed up for 76 months with no recurrence,while Case 2 died of recurrence and lung metastasis 3 months after sur-gery.Literature review suggested that pure epithelial NEN of the bladder exhibited typical hi

关 键 词：膀胱 纯的神经内分泌肿瘤 分化好的神经内分泌肿瘤 大细胞神经内分泌癌 

分 类 号：R737.14[医药卫生—肿瘤] R445[医药卫生—临床医学]