Fact-sheet: Cystic mesenchymal hamartoma of the liver
Updated on 09/17/2021 at 12:16 PM
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Definition
Very rare congenital condition Occurring in young children (< 2 years) Considered a hepatic malformation rather than a tumor, likely secondary to focal in utero hepatic ischemic phenomena Pathology: mixture of hepatocytes, bile ducts, vessels, and mesenchymal tissue. Theoretical risk of malignant transformation poorly understood.
Clinical features
Hepatomegaly
Laboratory findings
Normal (alpha-FP normal ++)
Ultrasound
Multiloculated, septated, well-defined cystic lesions without enhancement
Possible septal calcifications
No enhancement
Management
Surgical resection to be discussed at a Referral Center
Classification
Hepatic tumors in children
Differential diagnosis
Hepatic lesions in children:
Benign:
- Simple hepatic cysts (non-septated, anechoic content, older children)
- Multinodular hepatic hemangiomas (association with cutaneous involvement)
- Caroli disease/syndrome
- Von Meyenburg complexes
- Hepatic abscesses
Malignant:
- Hepatoblastoma: (solid tumor, markedly elevated Alpha-FP)
- Hepatic sarcoma: necrotic and mixed tissue features
- HCC: underlying liver disease