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Kidney Week

Abstract: TH-PO971

Fulminant Nephrotic Syndrome with Minimal Glomerular Findings Associated with a Pancreatic Neuroendocrine Tumor

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Lakhani, Laila, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Kyeso, Yousuf, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Sperati, John, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
Introduction

Neuroendocrine tumors (NETs) are a rare, heterogenous group of tumors that can present as benign or malignant lesions. Rare reports have associated NETs with membranous nephropathy. We report a patient with nephrotic syndrome and minimal glomerular findings who experienced clinical remission upon tumor resection.

Case Description

A 53 year old female with history of hypothyroidism presented with anasarca, 24-hour urine protein 4.2 g, hypoalbuminemia 2.1 g/dL, and acute kidney injury (serum creatinine 0.8 mg/dL increasing to 1.8 mg/dL). Kidney biopsy demonstrated normal glomeruli by light microscopy, mild tubular atrophy, and mild interstitial fibrosis. Immunofluorescence was unremarkable and electron microscopy revealed minimal foot process effacement with no electron dense deposits. Congo red staining was negative for amyloid. The acute kidney injury resolved. Eight months later, a pulmonary artery embolism was diagnosed, and she was noted to have 26 g on 24-hour urine protein collection. Serological evaluation for HIV, hepatitis C, hepatitis B, C3/C4, ANA, ANCA, SPEP, UPEP, serum free light chains, and PLA2R antibody were all normal/negative. UPCR decreased to 8 g/g on an ACEi. Six months later, UPCR again increased to 18 g/g, and repeat kidney biopsy demonstrated diffuse and moderate tubular injury, mild interstitial inflammation with clusters of eosinophils, and 25-30 % foot process effacement with no evidence of immune complex deposition. Abdominal MRI revealed an enhancing lesion in the tail of the pancreas that on resection was a well-differentiated, nonfunctional pT1N0 neuroendocrine tumor positive for chromogranin and synaptophysin with KI67 index <1%. One month after resection, urine dipstick protein was negative with albumin 3.5 g/dL, and 6 months later UPCR was 0.08 g/g, albumin 4.0 g/dL, and creatinine 0.9 g/dL.

Discussion

This case expands the association of NETs and nephrotic syndrome to include histologic lesions with minimal glomerular findings. The spontaneous remission with tumor resection supports the role of a circulating tumor-associated factor in the pathogenesis.