title: 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.
authors.affiliation: Paediatric Nephrology and Hypertension Unit, Obafemi Awolowo University Teaching Hospitals Complex, PMB 5538, Ile-Ife, Osun State, Nigeria. yetundeolowu@yahoo.com.
authors.forename: Wasiu Adekunle
authors.initials: WA
authors.lastname: Olowu
authors.affiliation: Urology Unit, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State, Nigeria.
authors.forename: Abdulkadr Ayo
authors.initials: AA
authors.lastname: Salako
authors.affiliation: Histopathology Department, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State, Nigeria.
authors.forename: Kayode Adebowale
authors.initials: KA
authors.lastname: Adelusola
authors.affiliation: Paediatric Surgical Unit, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State, Nigeria.
authors.forename: Oludayo Adedapo
authors.initials: OA
authors.lastname: Sowande
authors.affiliation: Radiology Department, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State, Nigeria.
authors.forename: Victor Adebayo
authors.initials: VA
authors.lastname: Adetiloye
authors.affiliation: Paediatric Nephrology and Hypertension Unit, Obafemi Awolowo University Teaching Hospitals Complex, PMB 5538, Ile-Ife, Osun State, Nigeria.
authors.forename: Olufemi
authors.initials: O
authors.lastname: Adefehinti
authors.affiliation: Histopathology Department, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State, Nigeria.
authors.forename: Stephen Adebayo
authors.initials: SA
authors.lastname: Osasan
mesh_terms: D058186:Acute Kidney Injury / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D006801:Humans; D006869:Hydronephrosis / Q000209:etiology / Q000473:pathology; D007668:Kidney / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D011507:Proteinuria / Q000209:etiology / Q000473:pathology; D018233:Rhabdomyosarcoma, Embryonal / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D016896:Treatment Outcome; D014513:Ureter / Q000473:pathology; D001743:Urinary Bladder / Q000473:pathology; D001749:Urinary Bladder Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D014567:Urography