Presentation
3 year old boy with recurrent episodes of macroscopic hematuria. Passed clot over the weekend. Patient in great deal of pain on admission . Nephritic screen normal. Renal function normal.
Patient Data
A 3 cm, well defined, multilobulated, echogenic mass is seen at the bladder base. The mass showed mild internal vascularity on color Doppler images. It was attached to the bladder wall, with no movement on decubitus views. There was no obvious extravesical extension within limitation of Ultrasound. An initial ultrasound diagnosis of an intravesical, non specific soft tissue mass attached to/arising from the bladder wall was made. Overt imaging features of malignancy not seen on ultrasound.
Case Discussion
The patient underwent cystoscopic biopsy at specialist Children's Hospital. A diagnosis of Embryonal Botryoid Rhabdomyosarcoma was made on biopsy
Pediatric urinary bladder masses are uncommon and majority are benign. The site of origin may be urothelial or mesenchymal. Rarely, other masses like paragangliomas can arise from chromaffin cell rests.
Imaging appearance is non specific and standard for diagnosis is cystoscopic biopsy.
Rhabdomyosarcoma is the commonest malignant mass. It may be embryonal or non- embryonal, with the latter having the worse prognosis.
Embryonal rhabdomyosarcomas could further be botryoid or alveloar. Botryoid variety can show 'bunch of grapes' appearance on imaging.
Work up for staging includes CT lungs, MR pelvis (for regional nodes), bone scan and PET scan, to exclude metastases to lungs, nodes or bones.
Chemotherapy is first line of treatment and usually curative. If it fails partial or total cystectomy may be needed. Annual surveillance on ultrasound to detect recurrence may be advisable.
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