Multimodality imaging in the diagnosis of biliary rhabdomyosarcoma in a young child: a case report.
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BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, but biliary involvement is extremely rare, accounting for approximately 0.5% of all pediatric RMS cases. CASE PRESENTATION: We report a 2-year-11-month-old girl presenting with obstructive jaundice, whose condition was initially misdiagnosed as choledochal cysts. Multimodality imaging-ultrasound, contrast-enhanced computed tomography (CT), magnetic resonance imaging (MRI)/ magnetic resonance cholangiopancreatography (MRCP), and positron emission tomography (PET)-CT-revealed a solid hilar mass with biliary dilation. This integrated imaging approach guided subsequent endoscopic retrograde cholangiopancreatography (ERCP) with stenting and open biopsy. Histopathology confirmed embryonal RMS (MyoD1+/Myogenin+). The patient was staged as intermediate-risk (stage III) and received vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy. CONCLUSION: Multimodality imaging is critical for timely diagnosis and staging of biliary RMS, enabling accurate biopsy and guiding multidisciplinary management.