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Abstract: SA-PO726

Ovarian Carcinoma Induced NELL-1 Membranous Nephropathy With Bilateral Renal Vein Thromboses

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials


  • Contreras, Cynthia, TriHealth, Cincinnati, Ohio, United States
  • Rajput, Amit K., TriHealth, Cincinnati, Ohio, United States
  • Abdul-Massih, Cesar Elias, TriHealth, Cincinnati, Ohio, United States
  • Samaha, Antoine L., TriHealth, Cincinnati, Ohio, United States

Membranous nephropathy (MN) is one of the most common causes of nephritic syndrome in adults, accounting for nearly one-third of all cases. PLA2R, a transmembrane receptor, is the major antigen in primary MN, found in approximately 70% of cases. Neural epidermal growth factor-like 1 (NELL-1) is seen in 16% of PLA2R negative MN. NELL-1 has a high association with underlying malignancy, seen in up to one-third NELL-1 positive MN patients.

Case Description

A 70-year-old female with a history of myelofibrosis was admitted by her nephrologist due to an asymptomatic acute kidney injury (AKI) with nephrotic range proteinuria (UPCR 23g). Her Cr increased from 1.1mg/dL to 2.5mg/dL in less than 3 months. Extensive serologic work up was unremarkable, prompting a renal biopsy. The renal biopsy revealed NELL-1 MN with 6/27 glomeruli globally sclerosed and severe arteriosclerosis. Interestingly, the biopsy also demonstrated focal glomeruli with rare intraluminal aggregates of fibrin material and focal glomerular hypercellularity, suggesting renal vein thrombosis.

During the work up, the patient was found to have a left renal vein acute thrombosis and no visualization of the right renal vein. A subsequent venogram demonstrated bilateral renal vein thrombosis, as well as thrombosis in the inferior vena cava and hepatic vein. The patient underwent multiple thrombectomies and thrombolytics, which improved her clot burden, but unfortunately was unable to salvage the right renal vein. The patient was started on long-term anticoagulation.

Given the association with NELL-1 MN and malignancy, the patient underwent an extensive work up. She was ultimately found with bilateral ovarian masses, which were consistent with high-grade serous carcinoma of the ovaries.


NELL-1 membranous nephropathy accounts for approximately 16% of all PLA2R negative MN cases. As seen with this patient, NELL-1 MN has a strong association with underlying malignancy. If diagnosed on biopsy, patients should undergo extensive cancer screening. As our understanding of this association progresses, more research is needed in guiding treatment for NELL-1 MN, especially cases with co-existing malignancies.