Abstract

Renal clear cell carcinoma (RCC), the predominant subtype of kidney cancer, is characterized by paraneoplastic syndromes, of which membranous nephropathy (MN) represents the most common paraneoplastic glomerulonephritis. While phospholipase A2 receptor (PLA2R) antibodies indicate primary MN, the concurrent presence of light-chain amyloidosis may further complicate the clinical manifestations in malignancy-associated cases. A rare case of RCC concurrent with phospholipase A2 receptor PLA2R-positive MN and light chain amyloidosis is reported in this paper. An elderly male patient presented with nephrotic syndrome and a right renal mass. The patient underwent a nephrectomy after admission. Pathological examination confirmed the diagnosis of right kidney renal clear cell carcinoma. Renal biopsy revealed PLA2R-positive MN and AL amyloidosis. Interestingly, in this case, glomerular light chain staining showed no monoclonal restriction, but λ light chain deposition was found in the vessels. However, the nephrotic syndrome showed no improvement after tumor resection. The patient achieved remission following sequential treatment with bortezomib, dexamethasone, daratumumab, and rituximab. This case illustrates the complex interplay among RCC, PLA2R-positive MN, and AL amyloidosis, complicating patient management. Future research should focus on the immunological mechanisms of PLA2R antibody production in malignancy and the pathophysiological links among these conditions. Clinicians should remain vigilant for secondary causes of nephrotic syndrome in renal malignancy patients and adopt comprehensive, multidisciplinary approaches to improve outcomes.


Author Details

Authors

• Jian Zhang¹,²
• Junyue Huang¹
• Bin Zhou³
• Limin Tian⁴,⁵
• Zhigang Ma⁶
• Wenhui Huang¹
• Hui Zhao¹,⁴

Departments

• ¹ Department of Nephrology, Gansu Provincial Hospital,
• ² The Second Hospital & Clinical Medical School, Lanzhou University,
• ³ The Blood Purification Center of Gansu Provincial Hospital,
• ⁴ College of Integrated Traditional Chinese and Western Medicine, Gansu University of Traditional Chinese Medicine, Lanzhou,
• ⁵ Department of Endocrinology, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu, and
• ⁶ Department of Nephrology, The Second Affiliated Hospital of The Chinese University of Hong Kong, Shenzhen (Longgang District People’s Hospital), Shenzhen, China

Address

Jian Zhang, 204 Donggang West Road Chengguan District, Lanzho 730000, Lanzhou, China
Email: [email protected]

Citation