CASE REPORT article
Front. Med.
Sec. Pulmonary Medicine
Volume 12 - 2025 | doi: 10.3389/fmed.2025.1610464
Giant Renal Angiomyolipoma Unmasks Underlying Lymphangioleiomyomatosis: A Case Report and Literature Review
Provisionally accepted
- 1Department of Urology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
- 2Institute of Urology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hebei Province, China
- 3Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Beijing, China
- 4Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
- 5Shenzhen Huazhong University of Science and Technology Research Institute, Shenzhen, China
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Lymphangioleiomyomatosis (LAM) is an uncommon systemic disease that primarily affects women during their reproductive years and is often linked with renal angiomyolipoma (AML). In this report, we describe a case involving a 30-year-old female patient who presented with a large AML in her right kidney. Imaging revealed diffuse pulmonary cysts, raising clinical suspicion of LAM. The procedure was initially planned as a minimally invasive partial nephrectomy performed with robotic assistance but was converted to open radical nephrectomy due to excessive intraoperative bleeding. Histopathological analysis confirmed epithelioid AML, and genetic testing revealed a somatic TSC2 mutation, further supporting the diagnosis of sporadic LAM. This case highlights the need to consider LAM in young women with renal AML and emphasizes the value of multidisciplinary management in addressing such complex clinical scenarios.
Keywords: Lymphangioleiomyomatosis, renal angiomyolipoma, Diagnosis and treatment, mTOR inhibitors, case report
Received: 06 Jun 2025; Accepted: 30 Jun 2025.
Copyright: © 2025 Zhou, Chen, Xiong, Wang and Zhang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Zhiyong Xiong, Department of Urology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
Miao Wang, Department of Urology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
Xiaoping Zhang, Department of Urology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
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