Abstract: TH-PO602
Rhabdomyosarcoma: A Frightening Differential for Bladder Outlet Obstruction (BOO) in Children
Session Information
- Trainee Case Reports - II
October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1600 Pediatric Nephrology
Authors
- Wei, Elizabeth, Warren Alpert Medical School, Providence, Rhode Island, United States
- Faizan, Mohammed Khurram, Hasbro Children's Hospital, Providence, Rhode Island, United States
- Kremsdorf, Robin Amy, Hasbro Children's Hospital, Providence, Rhode Island, United States
Introduction
Posterior urethral valves (PUV) are the most common cause of bladder outlet obstruction (BOO) in children. While many children are diagnosed prenatally on ultrasound, a few children may occasionally present outside of the neonatal period. In toddlers, bladder outlet obstruction can present with history of dribbling or weak urinary stream, and more often, following a febrile urinary tract infection. While PUV remains the most common cause of BOO in male children beyond the neonatal period, other etiologies for BOO must be considered, including urethral strictures, ureteroceles, bladder diverticula, and masses. We describe a case of rhabdomyosarcoma presenting with bladder outlet obstruction.
Case Description
13month old previously healthy full term Caucasian male presented with two weeks of constipation, decreased appetite, decreased urine output, and concern for dehydration. On exam, he had hypertension, a distended, tender abdomen, and normal external genitalia. Labs were notable for CO2 of 10mEq/dL, BUN 83mg/dL, creatinine 1.4mg/dL, and WBC 17.6 x109/L. Abdominal Xray was unremarkable. Ultrasound revealed a distended bladder and bilateral hydronephrosis consistent with BOO. Pt experienced significant clinical improvement following urethral catheter placement; his creatinine improved to 0.2mg/dL. On voiding cystourethrogram (VCUG) for evaluation of posterior urethral valves, he was found to have a 2.9 x 2.8cm lobulated prostatic mass with invasion into the bladder. Biopsy revealed embryonal rhabdomyosarcoma. At this time, he continues on chemotherapy and radiation therapy.
Discussion
Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma and accounts for 7-8% of all solid malignant tumors in children. It has a bimodal age distribution, affecting children less than two years old and adolescents. 15-20% arise from genitourinary sites. Bladder and prostate rhabdomyosarcoma can present with symptoms of bladder outlet obstruction, as in this patient. Prognosis and morbidity is dependent on staging, histology, age, and the extent of bladder sparing interventions. Rhabdomyosarcoma remains a rare, but important cause of bladder outlet obstruction in infants and toddlers. In children presenting with new onset obstructive uropathy, a broad differential including malignancy must be considered as prompt diagnosis allows for definitive therapy.