An antenatally diagnosed rhabdomyosarcoma of the bladder treated without extensive surgery

Abstract

Background. A female fetus was followed up from week 30 of gestation after bilateral hydroureteronephrosis and a large pelvic mass were detected on fetal imaging. At birth, the patient had high respiratory rate, a palpable bladder up to the umbilicus and a large pelvic mass, which compressed the anorectal wall.

Investigations. Fetal ultrasonography, fetal magnetic resonance urography, pelvic examination, full hematologic investigation, blood culture, abdominal and pelvic ultrasonography, voiding cystourethrography, abdominal and pelvic spiral CT, measurement of serum tumor marker levels, pathologic examination of the excised specimen, pelvic MRI.

Diagnosis. Botryoid subtype of rhabdomyosarcoma occupying most of the bladder and protruding through the urethra, with bilateral hydroureteronephrosis.

Management. Catheterization on the second day of life improved the patient's renal function, but her general condition remained unstable and surgical intervention was deferred. On the fourth day, gross hematuria and decreased urinary output were observed, which rapidly progressed to anuria, and she underwent transurethral resection of the protruding part of the tumor and bilateral cutaneous ureterostomy. Subsequently, she received chemotherapy with vincristine, actinomycin D and ifosfamide, and was followed up with serial imaging. At 18 months, MRI showed no evidence of residual tumor, and cystoscopic biopsy confirmed the absence of viable tumor; chemotherapy was stopped. She had no sign of recurrence 24 months after ending chemotherapy.