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Virtual Pediatric Hospital: Paediapaedia: Rhabdomyosarcoma of the Bladder Paediapaedia: Genitourinary Diseases

Rhabdomyosarcoma of the Bladder

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Symptoms are related to the growth of tumor into the bladder lumen and the proximal urethra. It is the most common soft tissue sarcoma in children, and has a peak incidence between 3-5 years old and between 15-18 years old.

Etiology/Pathophysiology:
Develops from primitive rhabdomyoblasts which are totipotential mesenchymal cells and is extremely malignant. It can arise anywhere in the body, but most commonly arises in the pelvis and genitourinary tract (39%) and in the head and neck (31%). There are three histological types: embryonal, alveolar, and pleomorphic. Embryonal and alveolar account for 90% of cases. Sarcoma botryoides is a type of embryonal that originates submucosally and then grows into the lumens of hollow organs. It grows rapidly, metastasizes early and wide with the exception of the embryonal type which infiltrates and recurs locally.

Pathology:
Not applicable

Imaging Findings:
It can appear as a solid infiltrative lesion or as a polypoid intraluminal mass that arises in the base of the bladder or in the region of the bladder trigone. Partial distal ureteral obstruction is often seen.

DDX:

References:

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