[Epithelioid angiomyolipoma of the kidney after successfully treated malignant melanoma].

Der Urologe. Ausg. A Pub Date : 2022-02-01 Epub Date: 2021-10-04 DOI:10.1007/s00120-021-01662-x
H Rothe, A Gaber, B Dittrich, M Nagel, M Tuffaha, B Hoschke
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引用次数: 1

Abstract

Background: Therapy of epitheloid angiomyolipomas (eAML) may be challenging, since unlike classical angiomyolipomas this rare subclass of benign mesenchymal angiomyolipomas may present with lymph node metastases, local recurrent disease, and/or systemic metastatic disease in up to 30% of cases.

Objectives: We report here for the first time in Germany a case of eAML after successful treatment of malignant melanoma.

Materials and methods: Clinical and histological findings as well as results of the genetic analysis of the angiomyolipoma are presented.

Results: A somatic, truncating mutation of the TSC2 gene was found in the angiomyolipoma.

Conclusion: The relationship to histologically similar tumor entities are presented and therapeutic options based on the genetic classification are discussed.

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【成功治疗恶性黑色素瘤后的肾脏上皮样血管平滑肌脂肪瘤】。
背景:上皮样血管平滑肌脂肪瘤(eAML)的治疗可能具有挑战性,因为与经典的血管平滑肌脂肪瘤不同,这种罕见的良性间充质血管平滑肌脂肪瘤亚类在高达30%的病例中可能表现为淋巴结转移、局部复发和/或全身转移性疾病。目的:我们在德国首次报道一例恶性黑色素瘤成功治疗后的eAML病例。材料与方法:报告血管平滑肌脂肪瘤的临床、组织学表现及遗传分析结果。结果:在血管平滑肌脂肪瘤中发现了TSC2基因的体细胞截断突变。结论:提出了与组织学上相似的肿瘤实体的关系,并讨论了基于遗传分类的治疗选择。
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