| Clinical features of infantile hepatic hemangioendothelioma |
| Eun Hee Kim (Kim EH), Kyung Nam Koh (Koh KN), Meerim Park (Park Mr), Bo Eun Kim (Kim BE), Ho Joon Im (Im HJ), Jong Jin Seo (Seo JJ) |
| Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Kore |
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Corresponding Author:
Eun Hee Kim ,Tel: +82-2-3010-3386, Fax: +82-2-473-3725, Email: luke2178@naver.com |
| Copyright © 2011 by The Korean Pediatric Society |
| ABSTRACT |
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Purpose : Infantile hepatic hemangioendothelioma (IHHE) is the most
common type of hepatic vascular tumor in infancy. We conducted this
study to review our clinical experience of patients with IHHE and to
suggest management strategies.
Methods : We retrospectively analyzed the medical records of 23 IHHE
patients (10 males, 13 females) treated at the Asan Medical Center
between 1996 and 2009.
Results : Median age at diagnosis was 38 days (range, 1 to 381
days). Seven patients (30%) were diagnosed with IHHE based on
sonographically detected fetal liver masses, 5 (22%) were diagnosed
incidentally in the absence of symptoms, 5 (22%) had congestive
heart failure, 3 (13%) had skin hemangiomas, 2 (9%) had abnormal
liver function tests, and 1 (4%) had hepatomegaly. All diagnoses were
based on imaging results, and were confirmed in three patients by
histopathology analysis. Six patients were observed without receiving
any treatment, whereas 12 received corticosteroids and/or interferonalpha.
One patient with congestive heart failure and a resectable
unilobar tumor underwent surgical resection. Three patients with
congestive heart failure and unresectable tumors were managed by
hepatic artery embolization with/without medical treatment. At a
median follow-up of 29 months (range, 1 to 156 months), 21 (91%)
patients showed complete tumor disappearance or >50% decrease in
tumor size. One patient died due to tumor-related causes.
Conclusion : IHHE generally has a benign clinical course with low
morbidity and mortality rates. Clinical course and treatment outcome
did not differ significantly between medically treated and non-treated
groups. Surgically unresectable patients with significant symptoms may
be treated medically or with hepatic artery embolization. |
| Keywords:
Infant | Liver | Hemangioendothelioma | Corticosteroid | Interferon-alpha | Therapeutic embolization
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