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Hemangioendothelioma of the hepatobiliary system: the classic and the unusual
M Maksimak1, E Wilt, A T Colley
1Department of Pediatric Subspecialties-Pediatric Gastroenterology, Geisinger Clinic, Danville, Pennsylvania 17822.
Insights
This study reports on two pediatric cases of hepatobiliary hemangioendothelioma. Successful treatment was achieved with steroids and surgery, highlighting effective management of this rare liver tumor.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Surgery
- Vascular Tumors
Background:
- Hepatobiliary hemangioendothelioma is a rare vascular tumor affecting the liver.
- Diagnosis and management can be challenging, particularly in infants.
Observation:
- A 7-month-old girl presented with hepatomegaly and heart failure, treated successfully with steroids.
- A 22-month-old boy had exclusive extrahepatic biliary tree involvement, presenting with jaundice.
Findings:
- The first patient showed excellent response to systemic steroids and supportive care.
- The second patient underwent successful surgical resection and Roux-en-Y biliary drainage, remaining asymptomatic for 2 years.
Implications:
- This case series demonstrates successful outcomes for pediatric hepatobiliary hemangioendothelioma.
- It highlights the efficacy of both medical and surgical interventions depending on tumor presentation.
Abstract:
Two children are reported with hemangioendothelioma of the hepatobiliary system. One patient, a 7-month-old girl, presented with classic symptomatology of hepatomegaly and congestive heart failure, but did not have any cutaneous lesions. She was treated with systemic steroids and supportive therapy and did very well. The other patient, a 22-month-old boy, is the first patient reported with exclusive involvement of the extrahepatic biliary tree. His symptoms included hepatomegaly and obstructive jaundice. He was evaluated with an endoscopic retrograde cholangiopancreatogram and a transhepatic cholangiogram. He did very well after resection of the lesion and Roux-en-Y drainage of the proximal biliary tree and remains asymptomatic 2 years after surgery.