Rhabdomyosarcoma accounts for 40% of paratesticular malignancies and 5% of testicular and paratesticular malignancies in children and adolescents. The Children's Oncology Group currently recommends computed tomography (CT) or magnetic resonance imaging for staging of paratesticular rhabdomyosarcoma in children. The present case illustrates a 9-year-old boy with paratesticular rhabdomyosarcoma who had negative findings on a staging CT scan and a subsequent positron emission tomography-CT scan demonstrating retroperitoneal lymph node metastasis. In the era of "as low as reasonably achievable" imaging, positron emission tomography-CT should be considered first-line imaging for staging to improve the sensitivity and specificity of staging for paratesticular rhabdomyosarcoma.
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