INFANTILE
HEMANGIOENDOTHELIOMA OF THE LIVER IN NEONATES
Trang-Tiau Wu*,
Meng-Cheng Chen, Wei-Chin Chiu
Department of
Pediatric Surgery, Chung-Shan Medical & Dental College Hospital, Taichung,
Taiwan, Chinese Taipei
Aim: Infantile Hemangioendotheliomas (IHE) are
the most common type of hepatic vascular tumors that present in infancy. We
reviewed our experience of I H E in terms of diagnosis, differential
diagnosis, surgical treatment, and outcome.
Materials and Results: Four infants (2 boys, 2
girls; age ranges: newborn- 2 months) were referred for definitive
management from 1991 through 2000. One neonate girl suffered from diffuse
neonatal hemangiomatosis over whole body skin surface and exhibited no
congestive heart failure and the bilobar multiple nodular lesion of liver
underwent spontaneous regression. Another three patients were symptomatic,
and the majority required intensive medical therapy because of anemia,
thrombocytopenia, respiratory distress and cardiac failure. All of three
symptomatic patients were treated by partial hepatectomy (L't hepatic lobe,
n = 3) for unilobar disease. We performed ultrasound, CT scan and MR I in
each one. At MR imaging performed with T2 - weighted pulse secuences the
lesion usually has high signal intensity. The Immunohistochemical study of
the endothelial cells of the tumors (n=3) is positive for factor VIII
related antigen. All are doing well except one patient died on respiratory
failure post-operatively after a mean follow-up period of 2.7 years.
Conclusions: IHE of the liver is a very rare
clinical entity. In our series study, if localized, hemangioendothelioma
can be radical hepatic resected , with rapid control of symptoms.