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Kidney Week

Abstract: FR-PO0682

Minimal Change Disease Associated with a Neuroendocrine Tumor of Unknown Primary

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Tiwari, Mahesh, Rutgers Robert Wood Johnson Medical School Department of Medicine, New Brunswick, New Jersey, United States
  • Fyfe-Kirschner, Billie S., Robert Wood Johnson University Hospital, New Brunswick, New Jersey, United States
  • Plamm, Alex, Robert Wood Johnson University Hospital, New Brunswick, New Jersey, United States
Introduction

Paraneoplastic minimal change disease (MCD) has been associated with hematologic, lung, renal, thymic malignancies and uncommonly neuroendocrine tumors (NETs) of duodenal or thymic origin. We present the first reported case of MCD associated with neuroendocrine neoplasm of unknown primary (NEN-UP).

Case Description

A 44-year-old man presented with a one-year history of an enlarging left neck mass. Core biopsy revealed well-differentiated grade 2 NET (chromogranin, synaptophysin, CAM5.2 and AE1/AE3 positive) with 5% Ki-67 index with proximal metastases. During admission for surgical debulking, he was found to have edema, weight gain, serum albumin 1.1 g/dL, urine albumin/creatinine 10,738 mg/g, cholesterol 469 mg/dL with creatinine 1.1 mg/dL at baseline. Lisinopril and loop diuretics were initiated, and a kidney biopsy was performed. Light microscopy and immunofluorescence of 34 glomeruli were unremarkable. Electron microscopy showed near-complete foot process effacement, consistent with MCD. One month later, he underwent surgical debulking and began prednisone 80 mg daily. Radiation started at two months and lantreotide 120 mg every 28 days began at three months (Figure 1). By four months, 24-hour urine protein decreased to 1.4 g (reference range 0-0.3 g). At ten months, 24-hour urine protein was 0.11g. He remains on lantreotide with steroid taper and no relapse.

Discussion

This is the first reported case of MCD associated with NEN-UP. Remission of MCD followed combined tumor-directed therapy (surgical debulking, radiation, and lantreotide) and corticosteroids. Clinicians should recognize MCD as a possible paraneoplastic glomerulopathy in NETs, including those without an identifiable primary site. Steroid use in this setting is controversial but should be considered when nephrotic syndrome is severe or malignancy directed treatment is delayed.

Figure 1: Remission of MCD after treatment of NET