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Infantile hepatic hemangioendothelioma with subsequent malignant degeneration
Insights
A rare angiosarcoma developed in a child previously treated for multiple hepatic hemangioendotheliomas. Long-term surveillance is recommended for children surviving liver hemangioendotheliomas to detect potential neoplastic disease.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Surgery
- Vascular Tumors
Background:
- Multiple hepatic hemangioendotheliomas are rare benign vascular liver tumors in infants and children.
- Steroid therapy is a common treatment for symptomatic hepatic hemangioendotheliomas.
- Long-term survival rates for hepatic hemangioendotheliomas have improved, necessitating consideration of late complications.
Observation:
- A 4-year-old child presented with an enlarging epigastric mass and anemia (falling hematocrit).
- The patient had a history of multiple hepatic hemangioendotheliomas treated with steroids 3.5 years prior.
- Imaging revealed a large, vascular mass in the left lobe of the liver.
Findings:
- Surgical resection (left hepatic lobectomy) was performed.
- Histopathological examination confirmed the diagnosis of angiosarcoma, a malignant vascular tumor.
- The angiosarcoma was found in the left lobe of the liver.
Implications:
- This case highlights the potential for malignant transformation (angiosarcoma) in children with a history of hepatic hemangioendotheliomas.
- Routine surveillance for neoplastic disease is crucial in pediatric patients who survive hepatic hemangioendotheliomas.
- Further research is needed to understand the risk factors and optimal surveillance strategies for these patients.
Abstract:
A 4 year old child was seen because of an enlarging epigastric mass and a rapidly falling hematocrit 31/2 years following steroid treatment for multiple hepatic hemangioendotheliomas. Technetium 99m sulfur colloid liver scan and hepatic angiography confirmed the presence of a large, vascular mass involving the left lobe of the liver. A left hepatic lobectomy was performed and histopathology showed angiosarcoma. As children with hemangioendotheliomas of the liver begin to survive for longer periods of time, surveillance for the possible development of neoplastic disease is recommended.