膀胱神经内分泌癌5例报告及文献复习

Zaouit Maryam, E. Asma, Oudad Fadwa, Jouhadi Hassan, B. Nadia, Sahraoui Souha, Benider Abdellatif
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引用次数: 0

摘要

原发性膀胱神经内分泌癌是一种具有明显转移潜力的侵袭性疾病。这是一种极为罕见的非尿路上皮性膀胱癌,占比< 1%。鉴于其罕见性,迄今为止尚未形成治疗共识。我们报告了五个回顾性的病例,并详细介绍了他们的解剖、临床和治疗方面。患者和方法:这是一项回顾性描述性研究,收集了2017年1月至2020年12月在穆罕默德六世中心收集的5例膀胱神经内分泌癌。经免疫组化检查确诊。中位年龄61岁(极值36-73岁),患者均为男性。咨询的平均延迟为7个月(极端:2-12个月)。3例患者长期吸烟。4例以血尿为主要征象。两例患者出现了刺激性泌尿系统疾病,如polakulia和排尿困难。膀胱镜检查显示多数病例为实体瘤,2例位于膀胱三角区,2例位于后基底壁。神经内分泌癌为纯癌4例,不纯癌4例;仅一例伴有低分化癌成分。膀胱小细胞神经内分泌癌的诊断是在免疫组织化学研究后保留的。所有病例均表达嗜铬粒蛋白A, 4例表达Synaptophysin。扩展检查发现3例患者(腹膜,肺,皮肤)局部晚期和立即转移性癌症。性能状态指数在0 ~ 1之间。3例患者推荐姑息性化疗,其中2例拒绝所有治疗。另外2例患者接受放疗作为新辅助化疗的辅助。结论:由于免疫组化诊断技术的进步,膀胱神经内分泌癌的发病率呈上升趋势。它们通常在局部已经晚期或转移的阶段被诊断出来;它们的演变非常迅速,预后很差。目前,对其治疗尚无共识。
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Bladder Neuroendocrine Carcinoma: About Five Cases and Review of the Literature
Introduction: Primary neuroendocrine carcinoma of the bladder is an aggressive disease with significant metastatic potential. It is an extremely rare variation of non-urothelial carcinoma of the bladder, accounting for < 1% [1]. Given its rarity, no therapeutic consensus has been codified to date. We report a retrospective series of five cases and detail their anatomoclinical and therapeutic aspects. Patients and methods: This is a retrospective descriptive study of five cases of neuroendocrine carcinoma of the bladder collected at the Mohammed VI Center, between January 2017 and December 2020. The diagnosis was confirmed by immunohistochemical study. The median age is 61 years (extremes: 36-73 years), all patients are male. The average delay of consultation is 7 months (extremes: 2-12 months). Chronic smoking was found in three patients. Hematuria was the cardinal sign in 4 cases. Irritative urinary disorders such as pollakiuria and dysuria were noted in two cases. Cystoscopy showed a solid tumor in the majority of cases, located in the bladder trigone in two patients, and in the posterobasal wall in two others. Neuroendocrine carcinoma was pure in four cases and impure; associated with a poorly differentiated carcinoma component in only one case. The diagnosis of small cell neuroendocrine carcinoma of the bladder is retained after an immunohistochemical study. Chromogranin A was expressed in all cases and Synaptophysin in four cases. The extension work-up revealed locally advanced and immediately metastatic cancer in three patients (peritoneum, lung, skin). The performance status index was between 0 and 1. Palliative chemotherapy was recommended in three patients, two of whom refused all treatment. The other two patients received radiotherapy as adjuvant to neoadjuvant chemotherapy. Conclusion: The incidence of neuroendocrine carcinomas of the bladder is increasing due to the diagnostic progress by immunohistochemical means available today. They are often diagnosed at a stage that is already locally advanced or metastatic; their evolution is very rapid and their prognosis is poor. At the present time, there is no therapeutic consensus for their management.
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