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Porta Hepatis Rhabdomyosarcoma
Case Detail
| Anatomy: Gastrointestinal |
 Joseph Junewick, MD FACR |
| Diagnostic Category: Neoplasia Malignant |
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| Created: over 9 years ago |
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| Updated: over 9 years ago |
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| Tags:
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| Modality/Study Types:
CT
US
NM
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Activities:  PDF  ImageJA |
History
2 year old male with jaundice.
Case Images
Diagnosis
Bilary Rhabdomyosarcoma
Findings
PET/CT – Infiltrative hypodense mass in the porta hepatis extending along the biliary ducts on CT and showing corresponding hypermetabolism on FDG imaging.
US – Hypoechoic mass in porta hepatis with dilated bile ducts.
Discussion
Biliary rhabdomyosarcoma occurs almost exclusively in children, diagnosed before the age of 5 years in most cases. Jaundice is the most common symptom although abdominal distention, fever, hepatomegaly, or nausea/vomiting may also be present. Laboratory examination usually reveals elevated bilirubin and alkaline phosphatase and normal serum AFP. Metastatic disease is present at diagnosis in up to a third of cases. Only the embryonal subtype of rhabdomyosarcoma occurs in the biliary tree. Rhabdomyoblasts infiltrate the subepithelial stroma of the biliary ducts. Immunohistochemical studies reveal positive reactions for muscle markers.
Reference
Chung EM, Lattin GE, Cube R, et al. Pediatric Liver Masses: Radiologic-Pathologic Correlation Part 2. Malignant Tumors. RadioGraphics 2011; 31:483–507.