WCN26-6678 SILENT INFLAMMATION IS LINKED TO ENDOTHELIAL DYSFUNCTION IN CHILDHOOD NEPHROTIC SYNDROME

Introduction: Nephrotic syndrome (NS) is occasionally associated with malignancy, most commonly with membranous nephropathy or minimal change disease (MCD) linked to Hodgkin's lymphoma.However, paraneoplastic NS secondary to ovarian or fallopian tube cancer is exceedingly rare.We report an autopsy-confirmed case of MCD associated with BRCA1 mutation-positive fallopian tube cancer in a patient with hereditary breast and ovarian cancer (HBOC) syndrome.Methods: A 77-year-old woman presented with generalized edema and proteinuria.She had a strong family history suggestive of HBOC-her daughter was BRCA1 mutation-positive, and her sister had breast cancer.Laboratory tests revealed nephrotic-range proteinuria (6.66 g/ gCr) and severe hypoalbuminemia (1.1 g/dL).Kidney biopsy showed normal glomeruli without immune deposits on immunofluorescence, and electron microscopy demonstrated diffuse podocyte foot-process effacement, confirming the diagnosis of MCD.She received intravenous methylprednisolone (1 g/day for 3 days), followed by oral prednisolone (40 mg/day) and cyclosporine (100 mg/day).Results: Despite combined corticosteroid and immunosuppressive therapy, nephrotic-range proteinuria persisted, and serum albumin levels remained markedly low.The patient's condition rapidly deteriorated due to massive edema, pleural effusion, and heart failure secondary to hypoalbuminemia, leading to death 41 days after admission.Autopsy revealed high-grade serous carcinoma of the right fallopian tube harboring a BRCA1 mutation, consistent with HBOC.No glomerulosclerosis or immune deposits were observed in the kidneys.These findings confirmed that the patient's MCD represented a paraneoplastic manifestation of fallopian tube cancer.Conclusion: This case represents the first reported association between fallopian tube cancer and MCD.In patients with steroid-resistant MCD, occult malignancy-including gynecologic cancers-should be considered.Genetic testing and risk-reducing salpingo-oophorectomy (RRSO) may help prevent such outcomes in BRCA1 mutation carriers.Early oncologic evaluation is essential when nephrotic syndrome fails to respond to standard immunosuppressive therapy.I have no potential conflict of interest to disclose.I did not use generative AI and AI-assisted technologies in the writing process.