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Hepatic hemangioendothelioma of infancy associated with elevated alpha fetoprotein and catecholamine by-products
1Department of Pathology, Wishard Memorial Hospital, Indiana University Medical Center, Indianapolis 46202.
Insights
Serum alpha-fetoprotein (AFP) levels are elevated in infants with hepatic hemangioendothelioma (HH). AFP levels correlate with age, decreasing as the infant gets older, and normalize after surgical resection of the tumor.
Area of Science:
- Pediatric Oncology
- Hepatology
- Tumor Biomarkers
Background:
- Hepatic hemangioendothelioma (HH) is a rare vascular tumor in infants.
- Understanding HH biomarkers is crucial for diagnosis and monitoring.
Purpose of the Study:
- To investigate the role of alpha-fetoprotein (AFP) and catecholamine by-products in infant hepatic hemangioendothelioma.
- To explore the relationship between AFP levels, age, and tumor characteristics.
Main Methods:
- Clinical chemistry analysis of serum markers.
- Immunohistochemical staining of tumor tissue.
- Electron microscopy for ultrastructural analysis.
Main Results:
- Elevated serum AFP levels were observed in four out of five infants with HH.
- The highest AFP levels were associated with younger infants and diffuse, unresectable tumors.
- AFP levels normalized in two patients following complete tumor resection.
- Elevated catecholamine by-products (VMA, HVA) were noted in one infant with extensive disease.
Conclusions:
- Serum AFP is a potential biomarker for hepatic hemangioendothelioma in infants.
- AFP levels in HH are inversely related to patient age and tumor resectability.
- The source of elevated catecholamine by-products may be stress-related rather than tumor-specific.
Abstract:
Five cases of hepatic hemangioendothelioma (HH) were seen in infants ranging from 2 days to 5 months of age. The cases were studied by clinical chemistry, immunohistochemical staining, and electron microscopic techniques. Serum alpha-fetoprotein (AFP) levels were elevated in each of the four patients in whom levels were obtained. The highest elevation was noted in a 2-day-old infant with a diffuse, unresectable lesion involving the right lobe. Two patients who underwent complete resections of their solitary HH had normalization of their AFP levels. This study suggests that AFP levels in patients with HH are closely related to the patients' age, with the youngest patient having the highest levels of AFP. Catecholamine by-products (VMA and HVA) were elevated in one of four patients in whom levels were obtained, this infant was 3 months old at the time of presentation. She also had an elevated AFP level, a diffuse unresectable lesion involving the entire liver, multiple pulmonary nodules, and cutaneous hemangiomas. Immunohistochemical study failed to demonstrate the source of AFP or the catecholamine by-products in the tumor of this patient, suggesting that the source of catecholamine by-products could be stress-induced catecholamine secretion.