肾移植术后肾周脂肪黏液样假性肿瘤3例的组织病理及分子特征分析。

IF 2.4 3区 医学 Q2 PATHOLOGY
Christina Schnoz, Marco Bonani, Florian Alexander Huber, Birgit Maria Helmchen, Thomas Fehr, Beata Bode-Lesniewska, Chantal Pauli, Ariana Gaspert
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引用次数: 0

摘要

背景:肾周黏液样假性脂肪瘤(PMPF)是一种少见的良性肾周及肾窦脂肪组织的假性肿瘤增生。其发病机制被认为是一个典型的与肿瘤和非肿瘤终末期肾脏疾病相关的反应过程。PMPF独特的组织病理学特征是一个粘液样过程,由温和的纺锤状基质细胞点缀着成熟的脂肪组织。在宏观上,它的特点是肿瘤脂肪瘤重塑的肾脏,这可能会引起恶性肿瘤的影像学关注。到目前为止,只有7例PMPF被记录在肾移植的背景下。病例介绍:本报告描述了三例肾移植后患者的PMPF,包括原生肾脏和移植肾脏。从宏观上看,所有病例均表现为肾脏萎缩,肾实质变薄萎缩,周围有大量增厚的肾周脂肪和团块形成的结节,这与术前的横断面成像结果一致。其余肾实质的组织学显示所有四个手术切除的肾脏终末期肾病,弥漫性间质纤维化,小管萎缩和肾小球硬化。肾周脂肪组织由成熟脂肪组成,具有明显的黏液样和胶原基质成分,散布着温和的梭形和星状细胞。S100、平滑肌肌动蛋白、desmin、IgG4免疫组化均为阴性。荧光原位杂交未发现MDM2基因扩增。使用FoundationOne®血红素测定法进行广泛的分子分析,未发现DNA和RNA水平致病性改变的证据。结论:PMPF是一种罕见的良性疾病,通常与慢性肾脏疾病相关,病程较晚。影像学表现可能被误认为恶性肿瘤,需要进行组织病理学检查以排除恶性肿瘤,特别是腹膜后的高分化或去分化脂肪肉瘤。肾移植受者可受PMPF影响,可在肾移植后数年发生在原生肾和移植肾中。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Perinephric myxoid pseudotumor of fat - histopathological and molecular characterization of 3 cases after renal transplantation.

Background: Perinephric myxoid pseudotumor of fat (PMPF) is a rare benign pseudo-neoplastic proliferation of the perinephric and renal sinus adipose tissue. Its pathogenesis is thought to be a reactive process typically associated with neoplastic and non-neoplastic end-stage kidney disease. The distinctive histopathological feature of PMPF is a myxoid process comprising bland, spindled stromal cells interspersed with mature adipose tissue. Macroscopically, it is characterized by tumorous lipomatous remodeling of the kidney, which may raise concerns of malignancy on imaging. To date, only seven cases of PMPF have been documented in the context of kidney transplantation.

Case presentation: This report describes three cases of PMPF in patients following renal transplantation, involving both native and grafted kidneys. Macroscopically, all cases consisted of shrunken kidneys with thinned and atrophic renal parenchyma surrounded by massively hypertrophic perirenal fat with mass-forming nodules, which was in concordance with cross sectional imaging findings acquired before surgery. Histology of the remaining renal parenchyma showed end stage renal disease in all four surgically removed kidneys, with diffuse interstitial fibrosis, tubular atrophy and sclerosed glomeruli. Perirenal adipose tissue consisted of mature fat with areas of significant myxoid and collagenous stromal component, interspersed with bland spindle and stellate-shaped cells. Immunohistochemistry for S100, smooth muscle actin, desmin and IgG4 were negative. No MDM2 gene amplification was identified by fluorescence in situ hybridization. Broad molecular profiling using the FoundationOne®Heme assay revealed no evidence of pathogenic alterations on DNA and RNA levels.

Conclusion: PMPF is a rare benign condition typically associated with chronic kidney disease, occurring late in the course. The radiological findings may be mistaken for those of a malignant tumor, and histopathological examination is required to exclude a malignant neoplasm, in particular a well-differentiated or dedifferentiated liposarcoma of the retroperitoneum. Renal transplant recipients can be affected by PMPF, which can occur in both native and transplanted kidneys several years following renal transplantation.

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来源期刊
Diagnostic Pathology
Diagnostic Pathology 医学-病理学
CiteScore
4.60
自引率
0.00%
发文量
93
审稿时长
1 months
期刊介绍: Diagnostic Pathology is an open access, peer-reviewed, online journal that considers research in surgical and clinical pathology, immunology, and biology, with a special focus on cutting-edge approaches in diagnostic pathology and tissue-based therapy. The journal covers all aspects of surgical pathology, including classic diagnostic pathology, prognosis-related diagnosis (tumor stages, prognosis markers, such as MIB-percentage, hormone receptors, etc.), and therapy-related findings. The journal also focuses on the technological aspects of pathology, including molecular biology techniques, morphometry aspects (stereology, DNA analysis, syntactic structure analysis), communication aspects (telecommunication, virtual microscopy, virtual pathology institutions, etc.), and electronic education and quality assurance (for example interactive publication, on-line references with automated updating, etc.).
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