Focal Nodular Hyperplasia with Steroid-Resistant Nephrotic Syndrome

Abstract

Background: Paraneoplastic glomerulonephritis (GN) are defined as glomerular lesions not directly related to tumor burden, invasion, or metastasis but induced by products of tumor cells. They are usually associated with solid tumors of gastrointestinal tract and hematological malignancies. Nephrotic syndrome is a rare manifestation of paraneoplastic syndromes, especially in children and biopsy findings are usually those of minimal change disease (MCD) or membranous nephropathy. We describe a case of steroid-resistant nephrotic syndrome with focal nodular hyperplasia (FNH) of liver. To the best of our knowledge, this has not been reported previously. Clinical Description: A 9-year-old boy presented with features of nephrotic syndrome and had a significant hepatomegaly. Based on radiological features and histopathological confirmation, the child was diagnosed to have a hepatic tumor consistent with a diagnosis of FNH, and the renal biopsy was suggestive of MCD. Management: As FNH did not require a surgical intervention, the nephrotic syndrome was managed with prednisolone initially, but the patient failed to achieve remission and was subsequently started on oral tacrolimus with enalapril and low-dose alternate day prednisolone. Remission of proteinuria was achieved within 2 months, and the child has been in follow-up for the past 18 months and the liver mass has been stable. Conclusion: This report suggests a possible paraneoplastic GN (steroid-resistant nephrotic syndrome) with FNH of liver that responded to immunosuppression.