Focal segmental glomerulosclerosis and nephrotic syndrome in a child with embryonal rhabdomyosarcoma

Abstract

A case of nephrotic syndrome (NS) and acute renal failure (ARF) associated with embryonal rhabdomyosarcoma (RMS) in a 10-year-old boy is reported. Ultrasound revealed irregular, echogenic, circumferential urinary bladder base mass, bilateral hydroureter and hydronephrosis. Histopathology of percutaneous renal and urethrocystoscopic biopsy specimens, respectively, revealed focal segmental glomerulosclerosis (FSGS) and embryonal RMS. Tumour remission was induced with pulse doses of intravenous vincristine, cyclophosphamide, methotrexate and actinomycin D over a 15-month period. He has been followed-up for 28 months and has maintained a drug-free tumour and proteinuria remission for 1 year. While some malignancies have been reported in association with NS, its occurrence in association with RMS is quite exceptional. We conclude that RMS may be associated with FSGS and NS. Effective treatment of the RMS was associated with sustained remission of the nephrotic proteinuria.

Rhabdomyosarcoma in a child with nephrotic syndrome treated with cyclosporine: a case report with literature review

“…We know of no other reports of RMS in children treated with CsA for nephrotic syndrome, although a case of embryonal RMS in pediatric nephrotic syndrome without evident immunosuppressive therapy has been described [8]. CsA has been widely accepted in the treatment of frequent relapsing nephrotic syndrome in children [9,10].…”