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Infantile mesenchymal hamartoma of the liver with elevated alpha fetoprotein
Liem Thanh Le1, Hai Thanh Phan1, Trung Sao Nguyen1
1Medic Medical Center, Ho Chi Minh, Vietnam.
Abstract:
Mesenchymal hamartoma of the liver (MHL) is a benign tumour that most commonly occurs in children. In most cases of MHL, the α fetoprotein (AFP) level is within the normal limits, only in a few cases, increased AFP has been described which usually causes misdiagnosis of hepatoblastoma. We report a case of a 3-month-old paediatric patient who was incidentally detected with a very high level of AFP, at 6388.4 ng ml-1. Ultrasound revealed a right liver tumour, segment VI, measuring at 56 × 53 mm. According to images of ultrasound and MRI, the diagnosis was mesenchymal hepatic sarcoma. The paediatric patient had surgery to remove the entire liver segment containing the tumour. Micropathological examination showed that the tumour was a MHL. The serum AFP level fell rapidly to near normal following the surgery. The MHL benign liver tumour with an atypical presentation caused a very high AFP level. This was a rare clinical case, and it was difficult to diagnose.
Insights
Mesenchymal hamartoma of the liver (MHL), a benign pediatric tumor, can present with unusually high alpha-fetoprotein (AFP) levels, mimicking malignancy. This case highlights the diagnostic challenge of MHL with atypical AFP elevation.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Pathology
- Diagnostic Imaging
Background:
- Mesenchymal hamartoma of the liver (MHL) is a rare, benign liver tumor predominantly seen in infants and young children.
- Elevated alpha-fetoprotein (AFP) levels are typically associated with malignant liver tumors like hepatoblastoma, posing a diagnostic challenge in differentiating benign conditions.
Observation:
- A 3-month-old infant presented with an incidentally detected liver mass and markedly elevated serum AFP levels (6388.4 ng/mL).
- Initial imaging (ultrasound and MRI) suggested mesenchymal hepatic sarcoma, a malignant diagnosis.
- Surgical resection of the liver segment containing the tumor was performed.
Findings:
- Post-operative histopathological examination confirmed the diagnosis of MHL, not mesenchymal hepatic sarcoma.
- Serum AFP levels rapidly normalized after surgical removal of the MHL.
- This case demonstrates an atypical presentation of MHL with significantly elevated AFP.
Implications:
- MHL should be considered in the differential diagnosis of pediatric liver masses with elevated AFP, even when levels are very high.
- Atypical presentations of MHL can lead to misdiagnosis and delayed treatment if not carefully evaluated.
- Accurate histopathological examination is crucial for definitive diagnosis and appropriate management of pediatric liver tumors.
