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肾脏碰撞瘤:三例附加病例报告。

Renal collision tumours: three additional case reports.

机构信息

Service d'Anatomie pathologique, CHU de Limoges, 2 avenue Martin Luther King Limoges cedex, 87042, Limoges, France.

Service de Radiologie et imagerie médicale, CHU de Limoges, 2 avenue Martin Luther King Limoges cedex, 87042, Limoges, France.

出版信息

BMC Urol. 2022 Jul 23;22(1):113. doi: 10.1186/s12894-022-01063-y.

Abstract

BACKGROUND

Multiple kidney tumours are frequently seen in hereditary syndromes and familial diseases. Renal collision tumours (RCT) are characterized by the simultaneous existence of different and unrelated tumour types within the same location in the kidney, forming a single, heterogenous lesion. RCT are uncommon histological entities with distinctive features. The most frequent subtypes include clear cell renal cell carcinoma (CCRCC), papillary renal cell carcinoma (PRCC), chromophobe renal cell carcinoma (CRCC), and collecting duct carcinoma (CDC).

CASE PRESENTATION

Here, we report three sporadic cases of RCT successfully treated by nephrectomy and confirmed by histological analysis. The first case was of a 64-year-old man diagnosed with RCT composed of a stage 2 nucleolar grade 3 CCRCC and a stage 1a nucleolar grade 2 type 1 PRCC. The second case was of a 68-year-old woman diagnosed with a combined nucleolar grade 2 type 1 PRCC and an angiomyolipoma (non-assessed stage), while the third case was of a 59-year-old woman diagnosed with a combined stage 1a nucleolar grade 3 CCRCC and a stage 1b CDC.

CONCLUSIONS

Due to the rarity of RCT, there are no standard guidelines for their management. Hence, the prognosis is considered to be associated with the most aggressive component, possibly the tumour with the highest nucleolar grade and stage. The histogenesis of RCT remains debated, and increase in knowledge regarding this can help enable the development of targeted therapies for advanced or metastatic tumours.

摘要

背景

多发性肾肿瘤在遗传性综合征和家族性疾病中经常可见。肾碰撞瘤(RCT)的特征是在肾脏的同一部位同时存在不同的、不相关的肿瘤类型,形成一个单一的、异质的病变。RCT 是一种罕见的组织学实体,具有独特的特征。最常见的亚型包括透明细胞肾细胞癌(CCRCC)、乳头状肾细胞癌(PRCC)、嫌色细胞肾细胞癌(CRCC)和集合管癌(CDC)。

病例介绍

在这里,我们报告了三例成功通过肾切除术治疗并通过组织学分析证实的散发性 RCT 病例。第一例是一名 64 岁男性,诊断为 RCT,由 2 期核仁 3 级 CCRCC 和 1 期 a 核仁 2 级 1 型 PRCC 组成。第二例是一名 68 岁女性,诊断为核仁 2 级 1 型 PRCC 合并血管平滑肌脂肪瘤(未评估分期),第三例是一名 59 岁女性,诊断为核仁 2 级 3 期 a CCRCC 合并 1 期 b CDC。

结论

由于 RCT 的罕见性,目前尚无其治疗的标准指南。因此,预后被认为与最具侵袭性的成分相关,可能是核仁分级和分期最高的肿瘤。RCT 的组织发生仍然存在争议,增加对其的认识有助于为晚期或转移性肿瘤开发靶向治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d64/9308929/3be3091298a4/12894_2022_1063_Fig1_HTML.jpg

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