Pediatric Pancreatic Hemangioma

Richard J England1, Helen Woodley2, Catherine Cullinane3,
Patricia McClean1, Jenny Walker4, Mark D Stringer1
1Children’s Liver and GI Unit, 2Department of Radiology, and 3Department of Pathology,
St. James’s University Hospital. Leeds, United Kingdom.
4Paediatric Surgical Unit, Sheffield Children’s HospitalSheffield, United Kingdom
ABSTRACT
Context The pancreas is an unusual site for a
hemangioma in an infant. A child with
obstructive jaundice caused by a pancreatic
hemangioma is presented and management
strategies for this benign tumor are discussed.
Case report A 5-month-old girl presented
with a 2-week history of jaundice, pale stools
and dark urine. Liver function tests confirmed
obstructive jaundice. An abdominal
ultrasound scan and magnetic resonance
imaging showed an enhancing mass in the
head of the pancreas. At laparotomy, a wedge
biopsy of the pancreatic tumor was taken and
a tube cholecystostomy inserted. Histological
examination of the specimen revealed a
pancreatic hemangioma with sclerotic
features. The high volume of bile loss from
the cholecystostomy proved problematic and
biliary diversion with a Roux-en-y
hepaticojejunostomy
was
therefore
performed. The tumor subsequently regressed
spontaneously and was no longer visible on
follow-up imaging two years later. The child
has since thrived.
Conclusions Pancreatic hemangiomas are
rare and may cause diagnostic confusion.
Pancreatic resection should be avoided since
the natural history of these benign tumors is
that of spontaneous involution. Various
strategies can be used to manage any
associated obstructive jaundice.
INTRODUCTION
Vascular anomalies can be divided into two
distinct categories, hemangiomas and vascular
malformations, based on clinical and
histological characteristics. Hemangiomas are
the most common tumor of infancy and
typically present as cutaneous lesions. Their
natural history consists of a proliferative
phase during the first year of life followed by
an involuting phase of variable duration
lasting up to 12 years of age [1].
Hemangiomas may cause cosmetic problems
or be complicated by ulceration and bleeding,
high output cardiac failure, Kasabach-Merritt
syndrome [2], or compression of adjacent
structures.
Visceral hemangiomas have been described in
various organs including the brain, parotid,
thorax, liver, spleen and gastrointestinal tract.
Pancreatic hemangiomas are rare. Less than
10 have been described in children. A
pancreatic hemangioma may present with
obstructive jaundice when it can lead to
diagnostic confusion and difficulties in
management. This report documents a further
case of an infantile pancreatic hemangioma

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and highlights some of the difficulties
encountered in clinical management.
CASE REPORT
A 5-month-old girl presented with a 2-week
history of jaundice, pale stools and dark urine.
She was born at term and had been otherwise
well. On examination she was markedly
jaundiced and had mild hepatomegaly but no
cutaneous hemangiomas. Biochemical liver
function tests were abnormal (total bilirubin
200 μmol/L, reference range: 5-21 μmol/L;
conjugated fraction 173 μmol/L, reference
range: 3-18 μmol/L; alanine aminotransferase
221 IU/L, reference range: 0-40 IU/L;
alkaline phosphatase 2,136 IU/L, reference
range: 100-400 IU/L; gamma glutamyl-
transpeptidase 957 IU/L, reference range: 0-
50 IU/L). Plasma lipase was mildly elevated
at 132 IU/L (reference range: 22-51 IU/L) but
plasma amylase was normal. Her hemoglobin
was 9.5 g/dL (reference range: 9-14.1 g/dL)
with a normal platelet count. Other
hematological investigations included a
normal immunoreactive trypsinogen, no
detectable common mutations for cystic
fibrosis, and normal alphafetoprotein and
alpha-1-antitrypsin levels. Urinary catechol-
amines were normal. An abdominal
ultrasound scan revealed a 3x2 cm mass in the
head of the pancreas but the echotexture of
the pancreatic head was similar to the
remainder of the gland. The common bile
duct was dilated (9 mm) and the gallbladder
distended with marked intrahepatic duct
dilatation. Magnetic resonance imaging
(MRI) including magnetic cholangio-
pancreatography (MRCP) confirmed a 3 cm
mass in the head of the pancreas with
associated biliary obstruction (Figures 1 and
2). The pancreatic duct in the body and tail of
the gland was not dilated but there was some
inflammatory change around the pancreas and
evidence of mild lymphadenopathy at the
porta hepatis and in the small bowel
mesentery. The mass showed delayed
enhancement after gadolinium injection with
centripetal filling (Figure 3).
A percutaneous needle biopsy of the tumor
was considered but it was decided to take an
open biopsy in order to obtain sufficient tissue
for histology. At laparotomy a hard, irregular
mass involving the head and neck of the
pancreas was identified. Some mesenteric
vascular congestion was noted. Several
Figure 1. Magnetic resonance cholangiopancreato-
graphy demonstrates a distended gallbladder and
dilated intra- and extrahepatic ducts down to the level
of the head of pancreas.
Figure 2. Coronal oblique MIP (maximum intensity
projection) post gadolinium image demonstrates a low
signal mass in the head of the pancreas causing nipping
of the portal vein.

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superficial wedge biopsies of the pancreatic
mass were taken along with a lymph node
from the porta hepatis. A tube
cholecystostomy was inserted.
Histopathology showed replacement of
pancreatic tissue by lobules of spindle cells
lining slit-like spaces and capillary sized
vascular channels (Figure 4). The lobules
were separated by dense fibrous tissue.
Immunohistochemistry showed the spindle
cells to be strongly positive for CD31 and
CD34 markers, consistent with an endothelial
origin (Figure 5). The stroma was strongly
positive for actin but negative for vimentin
and desmin. There was no evidence of
malignancy and the lymph node biopsy was
benign. It was concluded that the specimen
represented a pancreatic infantile capillary
hemangioma with marked sclerosis.
Cytogenetic analysis of the tumor showed a
normal female karyotype.
Postoperatively, bile drainage via the
cholecystostomy tube increased to 200-400
mL/day (maximum 3 mL/kg/h). During the
next two weeks, this fluid was replaced with
intravenous 0.9% sodium chloride and
potassium since the patient was unable to
tolerate full enteral feeding together with oral
fluid and electrolyte replacement. A
cholangiogram demonstrated persistent and
almost complete obstruction of the distal
common bile duct and a narrow, tortuous
cystic duct.
The patient’s bile losses precluded her
discharge home. Since it was anticipated that
it would take several months for her
hemangioma to show signs of spontaneous
involution an internal biliary bypass was
planned. She therefore underwent a
hepaticojejunostomy using a 35 cm Roux-en-
y loop of jejunum. Thereafter, she recovered
rapidly and uneventfully with complete
resolution of her jaundice. Two years later she
remains well with normal biochemical liver
function and no clinical or biochemical
evidence of pancreatic insufficiency. The
pancreatic mass is no longer detectable on
ultrasound imaging.
Figure 3. Coronal oblique post gadolinium image in
the portal venous phase (a.) demonstrates a low signal
mass in the head of pancreas with delayed
enhancement demonstrated on the delayed coronal
oblique post gadolinium acquisition (b.).
Figure 5. The lobules of capillaries are highlighted by
immunohistochemistry using the endothelial cell
marker CD34. Note the negative staining of pancreatic
glands to the left. (CD34 immunoreactivity, x100).
Figure 4. The biopsy shows lobules of small capillary
vascular channels separated by densely sclerotic stroma
replacing the pancreatic tissue. Some residual acinar
pancreatic tissue is seen in the upper right of the
specimen. (Hematoxylin and eosin, x100).

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DISCUSSION
Infantile hemangiomas, are vascular tumors
composed of blood vessels lined by
mitotically active endothelial cells. Unlike
vascular malformations which grow with the
individual, hemangiomas tend to undergo
proliferation during infancy followed by a
period of slow involution lasting several years
and eventual regression leaving a fibro-fatty
residuum [1]. They may occur in any region
of the body but have a predilection for the
head, neck and trunk. Treatment is variable
and a conservative approach is often justified
because of their natural history and benign
tendency. However, complications due to
their size or site may warrant medical or
surgical intervention. Visceral hemangiomas
may occur in isolation or coexist with
cutaneous lesions [3, 4].
Pancreatic hemangiomas in children are very
rare. Of more than 5,000 children in the
vascular anomalies database at the Children’s
Hospital Boston, only two had an infantile
hemangioma of the pancreas [5]. Only 9
children with a pancreatic ‘infantile’
hemangioma/hemangioendothelioma
have
been reported in the English literature (Table
Table 1. Pediatric pancreatic hemangiomas in the English literature.
Case Year
Authors
Age at presentation Sex
Presenting features
#1
1973
Chappell [6]
11 months
M
Jaundice, hepatomegaly
#2
1976
Tunell [7]
3 months
M
Jaundice, hepatosplenomegaly, abdominal
mass, Kasabach-Merritt syndrome
#3
1985
Horie et al. [8]
3 years
M
Jaundice, hepatomegaly
#4
1987
Sauer et al. [9]
5 months
F
Jaundice, hepatomegaly
#5
2003 Tebboune et al. [10]
2 months
F
Jaundice, hepatomegaly
#6
2003 Tebboune et al. [10]
18 months
M
Gastrointestinal bleeding, splenomegaly
#7
2005
Hibi et al. [11]
2 years
M
Jaundice
#8
2006
Vogel et al. [5]
2 months
F
Gastrointestinal bleeding
#9
2006
Vogel et al. [5]
2 years
M
Jaundice and abdominal pain
Table 1. (Continues)
Case
First line treatment
Subsequent treatment
Pathological description
#1
T-tube choledochotomy
Pancreaticoduodenectomy
Hemangioendothelioma
in head of pancreas
#2
Prednisone
Gastrojejunostomy and cholecystojejunostomy
(later reversed); radiotherapy
Hemangioendothelioma
in head of pancreas
#3
Choledochojejunostomy
None
Hemangioendothelioma
in head of pancreas
#4
Prednisone and biopsy
Prolonged percutaneous transhepatic biliary
stenting and external drainage
Hemangioendothelioma
in head of pancreas
#5
Percutaneous transhepatic
external biliary drainage
Hepaticojejunostomy
Hemangioendothelioma
in head of pancreas
#6
Percutaneous needle biopsy
and observation
None
Capillary hemangioma
in head of pancreas
#7
Percutaneous transhepatic
gallbladder drainage
Roux-en-Y choledochojejunostomy
Capillary hemangioma
in head of pancreas
#8
Corticosteroids
None
Infantile hemangioma
#9
Corticosteroids
Temporary percutaneous transhepatic external
biliary drainage
Infantile hemangioma

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1) [5, 6, 7, 8, 9, 10, 11] but the true incidence
of the condition may well be higher since
similar lesions in the body and tail of the
gland are likely to be asymptomatic. In these
reports, the terms hemangioma and
hemangioendothelioma appear to have been
used interchangeably to describe the same
clinical entity. Presenting features have
included obstructive jaundice, hepatomegaly,
a palpable mass, duodenal obstruction, and
intestinal bleeding. In one case, the lesion was
first detected by prenatal ultrasonography at
26 weeks’ gestation [10]. Cutaneous
hemangiomas were present in only one
patient [5]. All tumors were located in the
head of the pancreas. Table 1 does not include
children with more extensive retroperitoneal
hemangioendotheliomas involving multiple
organs including the pancreas, all of whom
also had the Kasabach-Merritt syndrome [4,
5, 12, 13, 14]. The latter is now recognized to
be a feature of the more aggressive
Kaposiform hemangioendothelioma rather
than the typical common infantile
hemangioma [2]. One other infant who died
from cardiac failure and was found at autopsy
to have multiple cutaneous and visceral
hemangiomas, including three on the surface
of the pancreas, was also excluded [3]. One of
the 9 children with an infantile hemangioma
arising from the pancreas (Table 1) had the
Kasabach-Merritt syndrome but it is difficult
to be certain about the exact histology of this
case reported in 1976 [7].
Ultrasound imaging of a pancreatic
hemangioma demonstrates a mass in the
pancreas frequently associated with biliary
obstruction. Portal vein stenosis and porto-
mesenteric vascular thrombosis have been
reported [8, 10]; our patient had extrinsic
compression of the portal vein (Figure 2).
MRI usually demonstrates more definitive
features of a hemangioma i.e. a lobulated
mass with a moderately high intensity signal
on T2 weighted images and marked
enhancement after intravenous gadolinium [5,
11]. MRCP sequences help to define
associated biliary obstruction. In our patient,
tumor enhancement after gadolinium
administration was delayed. This atypical
feature may have been due to the unusually
dense sclerosis within the tumor.
Conventional arteriography has been
described as an adjunct to diagnosis but does
not provide any additional useful information
unless selective embolization of feeding
vessels is being considered [5, 10].
The treatment of pancreatic hemangiomas in
childhood is variable and depends in part on
the presence of associated biliary obstruction.
Some tumors have been resected [4, 6] but
this is unnecessary. An adequate biopsy of
tumors at this site is important to eliminate
any doubt about the diagnosis. Treatment with
corticosteroids or alpha interferon may be
successful but tumor response to therapy is
variable [4, 5, 6, 9, 14]. Vincristine has been
used to treat the more aggressive Kaposiform
hemangioendothelioma
involving
the
pancreas [5]. One report described successful
percutaneous transhepatic biliary stenting and
external drainage in a 5-month-old infant
pending spontaneous tumor regression; the
infant required three catheter changes during
a 22-month period [9]. A similar attempt at
percutaneous transhepatic external drainage in
another infant had to be abandoned following
the development of septic complications and
the patient required an emergency
hepaticojejunostomy [10]. Other reports have
described internal surgical bypass procedures
using cholecystojejunostomy or choledocho-
jejunostomy [6, 7, 8, 11].
Our patient underwent an open biopsy of the
pancreatic mass to provide sufficient tissue
for diagnosis. This was considered especially
important because of the slightly atypical
features of the tumor on imaging. The
obstructive jaundice was initially managed by
a tube cholecystostomy which we hoped
would provide satisfactory external biliary
drainage. However, fluid and electrolyte
losses prevented this being a sustainable long
term option and a straightforward hepatico-
jejunostomy was therefore performed. This
avoided treatment with corticosteroids,
vincristine or interferon, all of which have a
significant risk of side-effects. If the expertise
is available, an alternative option worth
considering is temporary endoscopic biliary

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stenting but this would require regular stent
changes with the risk of cholangitis and post-
procedural pancreatitis. Cholecystojejuno-
stomy was considered in our patient but was
rejected for two reasons. Firstly, pancreatic
hemangiomas in the head of the pancreas can
cause cystic duct obstruction in infants [6, 9].
Secondly, we were concerned that our
patient’s narrow tortuous cystic duct might
cause problems in sustaining satisfactory
biliary diversion during the period of
involution of the hemangioma which may
take several years [6, 11].
In conclusion, pancreatic hemangiomas are
rare in children and often present with biliary
obstruction. The tumor does not require
resection because the natural history is that of
spontaneous involution. Associated biliary
obstruction can be managed in various ways
and, to some extent, the approach depends on
institutional expertise. In our patient, surgical
internal biliary diversion led to a good
outcome.
Received July 13th, 2006 - Accepted July 27th,
2006
Keywords
Hemangioma;
Jaundice,
Obstructive; Pancreas
Abbreviations MIP: maximum intensity
projection
Correspondence
Richard England
Department of Paediatric Surgery
Clarendon Wing
Leeds General Infirmary
Belmont Grove
Leeds, LS2 7TF
United Kingdom
Phone: +44-113.392.5077
Fax: +44-113.392.6609
E-mail: r.england@doctors.org.uk
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