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肾黏液性管状和梭形细胞癌黏液成分少,肾切除术后两年出现多处转移:一种罕见惰性肿瘤的非典型行为

Mucin-Poor Mucinous Tubular and Spindle Cell Carcinoma of the Kidney Presented with Multiple Metastases Two Years after Nephrectomy: An Atypical Behaviour of a Rare, Indolent Tumour.

作者信息

Sokolakis I, Kalogirou C, Frey L, Oelschläger M, Krebs M, Riedmiller H, Kübler H, Vergho D

机构信息

Department of Urology and Paediatric Urology, Julius Maximilian University of Würzburg, Würzburg, Germany.

Institute of Pathology, Julius Maximilian University of Würzburg, Würzburg, Germany.

出版信息

Case Rep Urol. 2017;2017:6597592. doi: 10.1155/2017/6597592. Epub 2017 Nov 14.

DOI:10.1155/2017/6597592
PMID:29348963
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5733991/
Abstract

BACKGROUND

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare type of renal cell carcinoma, whose clinical behaviour and metastatic potential have not been fully elucidated to date. There are only a few metastatic cases in the literature, which all either featured sarcomatoid differentiation or were synchronously metastasised at diagnosis.

CASE PRESENTATION

We report a case of a 49-year-old male with end-stage kidney disease on dialysis, presenting with multiple osseous metastases of a mucin-poor variant of MTSCC of the kidney, without sarcomatoid differentiation, two years after bilateral nephrectomy for papillary renal cell carcinoma (RCC) at a curable stage. After retrospectively reexamining the initial nephrectomy specimens, the tumour of the right kidney was also diagnosed as a mucin-poor variant of MTSCC, while the tumour of the left kidney was confirmed as a papillary RCC.

CONCLUSIONS

It is proposed that MTSCC can be associated with end-stage renal disease and that particularly the mucin-poor variant is easily confused with papillary renal cell carcinoma, as happened in this case. Although it is considered as a relatively indolent malign entity, it can metastasise even years after successful primary surgical treatment. This implies, besides accurate diagnosis, that MTSCC patients should be monitored closely in the follow-up period.

摘要

背景

黏液性小管状和梭形细胞癌(MTSCC)是一种罕见的肾细胞癌,其临床行为和转移潜能至今尚未完全阐明。文献中仅有少数转移病例,这些病例均具有肉瘤样分化特征或在诊断时同时发生转移。

病例报告

我们报告一例49岁男性终末期肾病患者,正在接受透析治疗,在因可治愈阶段的乳头状肾细胞癌(RCC)行双侧肾切除术后两年,出现了肾黏液缺乏型MTSCC的多处骨转移,且无肉瘤样分化。在对最初的肾切除标本进行回顾性复查后,右肾肿瘤也被诊断为黏液缺乏型MTSCC,而左肾肿瘤被确认为乳头状RCC。

结论

提出MTSCC可能与终末期肾病相关,特别是黏液缺乏型很容易与乳头状肾细胞癌混淆,本病例即如此。尽管它被认为是一种相对惰性的恶性实体,但即使在成功的初次手术治疗数年后仍可发生转移。这意味着,除了准确诊断外,MTSCC患者在随访期间应密切监测。

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