Related Experiment Videos
Hepatic hemangioendothelioma in children: analysis of thirteen cases
Chien-Chang Chen1, Man-Shan Kong, Chao-Ping Yang
1Department of Gastroenterology, Chang Gung Children's Hospital, No. 5, Fu-Hsin Street, Kuei-Shan, Taoyuan, Taiwan.
Insights
Hepatic hemangioendothelioma (HE) is a rare infantile liver tumor. While histologically benign, HE can lead to serious complications and poor outcomes, necessitating prompt diagnosis and management.
Area of Science:
- Pediatric Oncology
- Hepatology
- Vascular Tumors
Background:
- Hepatic hemangioendothelioma (HE) is a vascular tumor typically presenting in infancy.
- Clinical manifestations include hepatomegaly, abdominal mass, jaundice, and high-output cardiac failure.
Purpose of the Study:
- To review the clinical characteristics, diagnosis, management, and outcomes of hepatic hemangioendothelioma.
- To evaluate the effectiveness of various treatment modalities.
Main Methods:
- Retrospective review of 13 patients diagnosed with HE between July 1986 and June 2001.
- Diagnosis confirmed by histology or imaging studies (ultrasound, CT, MRI).
- Analysis of clinical manifestations, laboratory findings, and treatment outcomes.
Main Results:
- Common symptoms: abdominal distention (53%), congestive heart failure (38.5%), abdominal mass (30.8%), jaundice (30.8%).
- Hematologic abnormalities included anemia (53.8%), thrombocytopenia (30.8%), and coagulopathy (38.5%).
- Imaging revealed heterogeneous liver lesions; 4 out of 9 treated patients died from complications.
Conclusions:
- Hepatic hemangioendothelioma, though benign, can have a poor prognosis due to complications.
- Steroids are a first-line treatment, with other options including interferon, embolization, chemotherapy, and surgery.
- Long-term follow-up is crucial for assessing treatment response.
Abstract:
Hepatic hemangioendothelioma (HE) is a tumor that presents in infancy and toddler. It manifests hepatomegaly, abdominal mass, jaundice, abdominal distention, or high output cardiac failure. We reviewed patients with HE in our hospital in the past 15 years (from July 1986 to June 2001). The diagnosis was made by the histology specimen or various imaging studies. There were thirteen patients (9 males, 4 females) enrolled in our study. Their ages ranged from neonate to 2 years old. The common clinical manifestations included abdominal distention (53%), congestive heart failure (38.5%), abdominal mass (30.8%), jaundice (30.8%), and skin hemangioma (23.1%). Nine patients had serum alanine aminotransferase examination and were abnormal in 2. Anemia was noted in 7 of 13 (53.8%) patients, thrombocytopenia and hyperconsumptive coagulopathy were found in 4 and 5 patients, respectively. Serum alpha-fetoprotein was elevated in 4 of 7 patients. Abdominal ultrasonography (n = 13) showed heterogeneous and hypoechoic lesions in the liver. Computed tomography (n = 11) revealed central hypointensity with peripheral enhancement after contrast of the liver masses. Magnetic resonance imaging studies of the hepatic masses (n = 3) showed decreased signal intensity on T1 images and high signal intensity on T2. Most patients were treated with steroid. Other management included interferon, chemotherapy, embolization and/or surgery. Four patients were managed conservatively. Among the other nine patients, four patients died of sepsis, hepatic failure, disseminated intravascular coagulopathy or tumor rupture with hemorrhagic shock. HE appears to be a histologically benign tumor but may have a poor outcome because of complications. For its management, steroid is a first-line medication. Other methods of treatment were interferon, hepatic artery embolization, chemotherapy and surgery. Long term follow up is needed for the evaluation of treatment response.