Case report: Bilateral renal lymphangiomatosis with atypical manifestations in an elderly patient

Dingli Hu^1,2Yang Yuan³Bing Wang^1,2Yunlong Li^1,2*

• ¹Department of Urology, The First People’s Hospital of Kunshan, Suzhou,, Jiangsu, China
• ²Jiangsu University, Zhenjiang, China
• ³Panzhihua Municipal Central Hospital, Panzhihua, China

Renal lymphangiomatosis, also referred to as renal lymphangiectasia or renal lymphatic malformation, is a rare benign developmental disorder of the renal lymphatic system.[1] It is characterized by abnormal dilation of perirenal, hilar, and intraparenchymal lymphatic vessels, representing a malformative rather than a neoplastic process.[2] The precise pathogenesis remains incompletely understood; however, it is primarily attributed to congenital malformations of the renal lymphatic system, leading to impaired lymphatic drainage.[1] Other contributing factors may include localized inflammation, trauma, or extrinsic compression that mechanically disrupts normal lymphatic flow.[3] This condition can occur across all age groups and affects both sexes equally. Lesions may involve one or both kidneys. Most patients are asymptomatic; when present, symptoms are typically nonspecific and may include flank pain, hypertension, abdominal mass, hematuria, or lower extremity edema—often attributable to compression or traction effects from dilated lymphatic channels.[4] Imaging is the most reliable modality for diagnosing renal lymphangiomatosis.[2] Computed tomography (CT) is often considered the preferred diagnostic method due to its ability to clearly display cystic lesions and surrounding structures.[1] Bilateral renal involvement in renal lymphangiomatosis is particularly rare in elderly patients, and the total number of cases reported in the literature is limited. We present a rare case of bilateral renal lymphangiomatosis in a 63-year-old male presenting with atypical acute fever and left lower abdominal pain. Based on this case, we discuss the diagnostic approach, differential diagnoses, and management of renal lymphangiomatosis, with emphasis on recognizing atypical presentations to enhance clinical awareness of this rare entity.