A case of large renal angiomyolipoma resulted in hydronephrosis.

IF 1 4区医学 Q4 PATHOLOGY

Malaysian Journal of Pathology Pub Date : 2025-08-01

W M Phang, J W D Tan, E H Goh, L L Goh, Y P Wong, G C Tan

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引用次数: 0

Abstract

Introduction: Renal angiomyolipoma is one of the members of the perivascular epithelioid cell (PEC) tumour family. It has a characteristic triphasic morphology featuring varying proportions of dysmorphic blood vessels, smooth muscle cells, and mature adipose tissue. Large angiomyolipomas pose a risk of haemorrhagic complications.

Case report: A 47-year-old woman presented with right abdominal pain for 2 months, and radiological investigation revealed a 14.2 cm renal mass causing hydronephrosis. The symptoms persisted after arterial embolisation and hence, nephrectomy was performed. The excised renal tumour weighed 1,442 grams. There was a well-circumscribed yellow mass confined within the Gerota's fascia. Histological examination revealed a classic triphasic morphology comprising mature adipose tissue, dysmorphic blood vessels, and smooth muscle. Notably, there were post-embolisation histological changes in extensive fat necrosis and foreign body giant cell reaction. Immunohistochemically, it expressed HMB45, MelanA, and SMA, while it was negative for PAX8 and pan-cytokeratin.

Discussion: We described a case of renal angiomyolipoma with classic gross appearance of adipose-rich tumour and the triphasic histological features and discussed the post-embolisation histological changes.

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大肾血管平滑肌脂肪瘤致肾积水1例。

肾血管平滑肌脂肪瘤是血管周围上皮样细胞（PEC）肿瘤家族的成员之一。它具有典型的三相形态，以不同比例的畸形血管、平滑肌细胞和成熟脂肪组织为特征。大血管平滑肌脂肪瘤有出血并发症的风险。病例报告：一名47岁女性，以右腹痛2个月为主诉，影像学检查发现一14.2 cm的肾肿块，引起肾积水。动脉栓塞后症状持续，因此行肾切除术。切除的肾肿瘤重1442克。在Gerota的筋膜内有一个界限清楚的黄色肿块。组织学检查显示典型的三相形态，包括成熟的脂肪组织、畸形的血管和平滑肌。值得注意的是，栓塞后存在广泛的脂肪坏死和异物巨细胞反应的组织学改变。免疫组化表达HMB45、MelanA、SMA， PAX8、泛细胞角蛋白阴性。讨论：我们报告了一例肾血管平滑肌脂肪瘤，具有典型的富含脂肪肿瘤的大体外观和三相组织学特征，并讨论了栓塞后的组织学变化。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Malaysian Journal of Pathology PATHOLOGY-

CiteScore

3.60

自引率

5.60%

发文量

期刊介绍： The Malaysian Journal of Pathology is the official journal of the College of Pathologists, Academy of Medicine Malaysia. The primary purpose of The Journal is to publish the results of study and research in Pathology, especially those that have particular relevance to human disease occurring in Malaysia and other countries in this region. The term PATHOLOGY will be interpreted in its broadest sense to include Chemical Pathology, Cytology, Experimental Pathology, Forensic Pathology, Haematology, Histopathology, Immunology, Medical Microbiology and Parasitology. The Journal aims to bring under one cover publications of regional interest embracing the various sub-specialities of Pathology. It is expected that the articles published would be of value not only to pathologists, but also to medical practitioners in search of a scientific basis for the problems encountered in their practice, and to those with an interest in diseases which occur in the tropics.