sarcoma botryoides

Sarcoma Botryoides Presenting as Urethral Prolapse

Cohen Children’s Medical Center of New York,
New Hyde Park

sarcoma botryoides

Following an episode of hematuria and passage of debris in the urine, a 16-month-old Caucasian girl was brought to the emergency department (ED). There was no report of fever or history of trauma. The parents are the only caregivers. The child had been treated with oral antibiotics for 2 prior urinary tract infections. She had 1 previous episode of hematuria; at that time, urethral prolapse was diagnosed and a topical estrogen cream was prescribed.

The patient was well-appearing. Vital signs were normal. General physical and systemic findings were all normal. The abdomen was nondistended, with no palpable masses. Genitourinary examination revealed a doughnut-shaped, erythematous, circumferential mass around her urethra—a classic finding of urethral prolapse.

Persistent dribbling of urine from the mass into her diaper was noted. This finding and the patient’s young age prompted further work-up with an ultrasound examination. This revealed a large multiloculated cystic mass in the bladder that was later identified as sarcoma botryoides.

Urethral prolapse most commonly occurs in young prepubertal African-American girls (peak incidence 4 to 10 years) and in postmenopausal Caucasian women. The usual presentation is vaginal bleeding or bloodstains on the child’s underwear, which often leads to suspicion of child abuse.1,2 Hematuria is uncommon.

Sarcoma botryoides, or embryonal rhabdomyosarcoma, has been reported to arise from the vagina, urinary bladder, common bile duct, and nasopharynx. Although rare, it is the most common vaginal cancer in children. This tumor usually occurs in girls younger than 8 years, with a peak incidence at age 3 years. The term “botryoides” comes from the Greek word botrys for grapes and describes the grape-like appearance of the tumor.3

This patient had a rare form of sarcoma botryoides, with the tumor arising from the bladder wall. The tumor location may explain the constant urinary dribbling, hematuria, and increased bladder pressure that resulted in urethral prolapse. Patients with vaginal tumors may also present with a polypoid mass protruding from the introitus that can be mistaken for urethral prolapse. Surgical resection along with radiation and chemotherapy is usually indicated.4,5 The girl in this case underwent radiation therapy and was scheduled for surgery at a later date.

The clinician must recognize atypical cases of urethral prolapse and look for precipitating factors. Urethral prolapse that occurs in a child outside the usual age range at presentation or with atypical findings should raise the index of suspicion for a genitourinary mass. Ultrasound examination should be considered in the evaluation. Prompt diagnosis and early consultation with the appropriate specialist will lead to early treatment and improved outcomes for the patient.