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2型乳头状肾细胞癌之前的局灶节段性肾小球硬化

Focal Segmental Glomerulosclerosis Preceding Type 2 Papillary Renal Cell Carcinoma.

作者信息

Dabrowski Dominik, Ozluk Ekin, Barbeito Silvia, Wei Eric X

机构信息

Department of Pathology and Translational Pathobiology, LSU Health Shreveport, USA.

Department of Radiology, LSU Health Shreveport, USA.

出版信息

Case Rep Pathol. 2020 Oct 15;2020:8811905. doi: 10.1155/2020/8811905. eCollection 2020.

Abstract

Renal cell carcinoma (RCC) is the predominant renal malignancy in adults. Of the four general subtypes, papillary renal cell carcinoma (P-RCC) is the second most common and can be subdivided into type I, type II, and a mixture of type I and II. Focal segmental glomerulosclerosis (FSGS) is the most common glomerulopathy at all ages, and it can be seen as a paraneoplastic syndrome. RCC, in general, is known to present with many paraneoplastic syndromes, and glomerulopathies are among these. Rarely, RCC and glomerulopathies may overlap in the same patient. Here, we report a 58-year-old male with a past medical history of FSGS and chronic kidney disease (CKD), stage III, who was found to have an incidental renal mass that was later diagnosed as type II P-RCC. The histology showed pseudostratified tumor cells with an eosinophilic cytoplasm that formed papillary configurations and displayed areas of necrosis. The prior FSGS diagnosis exhibited segmental sclerosis, refractory tufts, and capillary membrane wrinkling. A period of 1.5 years elapsed between the diagnosis of the glomerulopathy and the malignancy. The tumor was found to be at stage TIb. To our knowledge, this may be the first reported case of usual-type FSGS as paraneoplastic glomerulopathy (PG) preceding P-RCC. Because FSGS only sparingly affects the kidney and is a common glomerulopathy in adults, it is reasonable to complete comprehensive diagnostic studies and commence medically necessary treatment, especially in the background of other renal comorbidities. These preexisting comorbidities may be associated with malignancy very early in its course. The probability of RCC-associated paraneoplastic glomerulopathy is low, which means an already incidentally found renal mass may conceal a serpentine paraneoplastic syndrome. A more developed understanding of these manifestations can lead experienced clinicians to suspect and possibly uncover an insidious RCC before it advances.

摘要

肾细胞癌(RCC)是成人中主要的肾脏恶性肿瘤。在四种常见亚型中,乳头状肾细胞癌(P-RCC)是第二常见的类型,可细分为I型、II型以及I型和II型的混合类型。局灶节段性肾小球硬化(FSGS)是各年龄段最常见的肾小球病,可被视为一种副肿瘤综合征。一般而言,RCC已知会出现多种副肿瘤综合征,肾小球病也在其中。很少有RCC和肾小球病会在同一患者中重叠。在此,我们报告一名58岁男性,既往有FSGS和慢性肾脏病(CKD)III期病史,偶然发现肾脏肿物,后来被诊断为II型P-RCC。组织学显示假复层肿瘤细胞,胞质嗜酸性,形成乳头状结构,并可见坏死区域。先前的FSGS诊断表现为节段性硬化、难治性肾小球小叶和毛细血管膜皱缩。从肾小球病诊断到恶性肿瘤诊断间隔了1.5年。肿瘤被发现处于TIb期。据我们所知,这可能是首例报道的在P-RCC之前出现的普通型FSGS作为副肿瘤性肾小球病(PG)的病例。由于FSGS仅轻度累及肾脏且是成人常见的肾小球病,因此完成全面的诊断性检查并开始进行必要的医学治疗是合理的,尤其是在存在其他肾脏合并症的情况下。这些先前存在的合并症可能在恶性肿瘤病程的早期就与之相关。RCC相关的副肿瘤性肾小球病的概率较低,这意味着偶然发现已经存在的肾脏肿物可能隐藏着一种隐匿的副肿瘤综合征。对这些表现有更深入的了解可以使经验丰富的临床医生在隐匿性RCC进展之前就怀疑并可能发现它。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e18f/7585650/20fd495e63b8/CRIPA2020-8811905.001.jpg

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