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Elevated serum a-fetoprotein in a neonate with cutaneous infantile
hemangioendothelioma
Tai-Heng Chen,
1,3,5
Jan-You Lin,
4
Wu-Fu Chen,
6
Yue-Chiu Su
2
and San Nan Yang
1,5
Departments of
1
Pediatrics, and
2
Pathology,
3
Division of Pediatric Emergency, Department of Emergency,
4
Division of
Pediatric Surgery, Department of Surgery, Kaohsiung Medical University Hospital, and
5
Graduate Institute of Medicine,
Kaohsiung Medical University, and
6
Department of Neurosurgery, Chang Gung Memorial Hospital – Kaohsiung Medical
Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan
Key words a-fetoprotein, extrahepatic, hemangioendothelioma, neonatal.
Infantile hemangioendotheliomas (IHE) are rare vascular tumors
occurring mainly during infancy.
1
Approximately 85% of cases
present before 6 months of age with predominant involvement of
the liver, lung, calvarial bones or soft tissues of limbs.
2,3
In contrast
to asymptomatic hemangiomas, which usually undergo spontane-
ous regression within 1 year, IHE may be rapidly fatal due to
complications of congestive heart failure, consumptive coagul-
opathy, and respiratory compromise, resulting in a mortality rate
as high as 90%.
1,3,4
Patients with hepatic IHE are occasionally
found to have a high serum a-fetoprotein (AFP) level,
1,5
but have
never been reported in solely cutaneous or soft-tissue-involving
IHE. In addition, the recurrence of IHE is uncommon.
3
Here, we
report a neonate with recurrent non-visceral IHE with persistently
elevated serum AFP level, despite complete initial surgical exci-
sion of the tumor. We suggest AFP level to be a useful clinical
index for monitoring the bioactivity of extrahepatic IHE.
Case Report
A 14-day-old male neonate presented to our clinic with a 5 ¥
4-cm
2
mass noted at his right medial thigh since birth. He was
delivered vaginally at 39 weeks of gestation with a birth body-
weight of 3038 g to nonconsanguineous parents. His antenatal
ultrasonographic findings were negative and the perinatal period
was uneventful. The mass appeared to be soft, flesh-colored with
scattered violaceous stains, non-pulsatile and completely covered
with skin (Fig. 1). There were no signs of hepatosplenomegaly or
Correspondence: San Nan Yang, MD PhD, Department of Pediatrics,
Kaohsiung Medical University Hospital, Kaohsiung Medical Univer-
sity, No. 100, Tzyou 1st Road, Sanmin District, Kaohsiung City 807,
Taiwan. E-mail address: y520729@gmail.com
All authors of this article indicated no potential conflicts of interest.
Received 8 May 2009; revised 19 May 2010; accepted 2 August
2010.
doi: 10.1111/j.1442-200X.2010.03242.x
Fig. 1 The 5 ¥ 4-cm
2
protruding mass, located over the medial part
of the right thigh. On gross examination, the mass appeared flesh-
colored with several violaceous stains and was covered with intact
skin without ulceration.
258 K Miyako et al.
© 2011 The Authors
Pediatrics International © 2011 Japan Pediatric Society