Hamartomatous Polyp of the Gallbladder With an Associated ...

Hamartomatous Polyp of the
Gallbladder With an Associated
Choledochal Cyst
Abbreviations
PJS, Peutz-Jeghers syndrome
Received May 24, 2010, from the Department of
Radiology, Division of Abdominal Imaging and
Intervention, Massachusetts General Hospital,
Harvard Medical School, Boston Massachusetts
USA. Revision requested June 7, 2010. Revised
manuscript accepted for publication July 1, 2010.
Address correspondence to A. Devrim
Karaosmanoglu, MD, Department of Radiology,
Division of Abdominal Imaging and Intervention,
Massachusetts General Hospital, Harvard Medical
School, 55 Fruit St, White 2, Boston, MA 02114 USA.
E-mail: alidevrim76@yahoo.com
A. Devrim Karaosmanoglu, MD, Michael Blake, MD
hamartomatous polyp is an extremely rare polypoidal lesion of the gallbladder,
with the first case having been reported in 1998. 1 To the best of our
knowledge, fewer than 10 hamartomatous gallbladder polyps have been
reported in patients with Peutz-Jeghers syndrome (PJS). The largest series
evaluating extraintestinal polyps in patients with PJS revealed 3 patients with a gallbladder
polyp among 72 patients with 1 pathologically proven hamartomatous polyp. 2
A
Hamartomatous gallbladder polyps are thus rare in patients with PJS, and as mentioned
before, to the best of our knowledge, it has not been reported before in a patient
without PJS. Another interesting finding in this case was the association of a type 1
choledochal cyst with the hamartomatous gallbladder polyp.
Case Report
Case Report
A 16-year-old male patient presented with right upper
quadrant pain and a mild increase in transaminases. His
medical and family histories were unremarkable for any
notable medical problem. A sonographic examination
of the patient showed a nonshadowing 4 × 3-cm slightly
hyperechoic mass in the gallbladder, almost completely
filling the lumen (Figure 1). The mass was a broad-based
lesion with intense vascularization on a color Doppler
study (Figure 2). The lesion did not show any frank invasion
into the adjacent liver tissue, and the tissue planes
between the mass and the gallbladder wall were intact.
There was also dilatation of the common hepatic duct
and the common bile duct, most notable in the superior
two-thirds.
The patient was then referred for computed tomography,
which showed a contrast-enhancing mass in the
gallbladder with no evidence of liver invasion and dilatation
of the common hepatic duct and the upper twothirds
of the common bile duct (Figure 3). Endoscopic
retrograde cholangiopancreatography then confirmed
the diffuse dilatation of the common hepatic and common
bile ducts with no evidence of a mass or stone, consistent
with a type 1 choledochal cyst (Figure 4).
© 2010 by the American Institute of Ultrasound in Medicine J Ultrasound Med 2010; 29:1663–1666 0278-4297/10/$3.50

Hamartomatous Polyp of the Gallbladder With Choledochal Cyst
1664
Figure 1. Sagittal sonogram of the gallbladder showing a slightly
hyperechoic solid mass (arrows) originating from the fundal
wall of the gallbladder and projecting inferiorly toward the neck
of the gallbladder. No definite evidence of extension into the
liver parenchyma is shown.
Given these findings, the patient underwent a
cholecystectomy and resection of the extrahepatic
bile ducts with a hepaticojejunostomy.
Pathologic imaging showed an exophytic graytan-red
mass attached to the mucosal surface of
the gallbladder with no evidence of liver invasion,
and the microscopic analysis characterized
this lesion as a hamartomatous polyp. The
resected extrahepatic bile ducts showed dilatation
compatible with a choledochal cyst with no
evidence of malignancy or an obstructive lesion.
The postoperative period was uneventful, and
the patient was completely healthy with no evidence
of disease at a 4-year follow-up. Clinical
examination, upper gastrointestinal barium
study, and small-bowel follow-through examination
findings, as well as the family history, were
all negative.
Discussion
Gallbladder polyps are common and well-known
abnormalities in the general population. By definition,
any lesion protruding from the wall of the
gallbladder into the lumen is called a polyp. The
incidence of detection varies between 5.6% and
6.9% in healthy patients who undergo sonography.
3,4 Gallbladder polyps may be grossly divided
into 2 groups as tumorous and nontumorous
polyps. The nontumorous group consists of
inflammatory and cholesterol polyps, whereas
tumorous polyps are adenomas, adenomyomas,
and early gallbladder carcinomas. 5 The medical
literature is contradictory for the management of
these polyps, but the current overall consensus is
to surgically remove all gallbladder polyps of 10
mm or larger. 6 Most polyps detected on sonography
are nontumorous polyps. Although several
imaging clues may favor one or the other, no
Figure 2. Axial (A) and sagittal (B) color Doppler images showing intense vascularization (arrows) within the lesion, confirming that
the mass is solid and contains viable tissue.
A B
J Ultrasound Med 2010; 29:1663–1666

A B
Figure 3. Axial (A) and coronal (B) intravenous contrast agent–enhanced computed tomograms. A, Solid mass in the gallbladder
showing substantial contrast enhancement (arrows). B, The visualized common hepatic duct and upper third of the common bile duct
are dilated with mild prominence of central intrahepatic bile ducts (thin arrow). The mass in the gallbladder is shown again (thick
arrow).
finding is specific for malignant or premalignant
lesions of the gallbladder. Age older than 50
years, the presence of gallstones and underlying
primary sclerosing cholangitis, a rapid increase
in size, and the presence of a broad base are all
considered risk factors for malignancy in the setting
of a gallbladder polyp. 5 Gallbladder carcinoma
is rarely detected as small polypoid lesions
on routine imaging studies and, unfortunately, is
usually diagnosed at late stages when a curative
surgical treatment is not an option.
By definition, hamartoma means tumoral malformation
of orthotopic tissue. Hamartomatous
polyps of the gallbladder are extremely rare
abnormalities and were first reported in 1998 1 in
a 2-year 6 month-old boy with no known history
of PJS at the report of the case. The presence
of gallbladder polyps is an uncommon event in
the pediatric population, and only 11 patients
with a primary polypoid lesion of the gallbladder
were reported up until 2003. 7 The abnormalities
detected in the pediatric age group in
that study included papillary hyperplasia, a
cholesterol polyp, adenoma, and gastric heterotopias.
7 Peutz-Jeghers syndrome may be
counted among the secondary causes of gallbladder
polyps, along with pancreaticobiliary
maljunction and metachromatic leukodystrophy.
8 Hamartomatous polyps are also not common
in PJS, and in a study of 72 patients with PJS,
only 3 were found to have gallbladder polyps.
In that study, 2 of the patients were followed
without any intervention, and only 1 patient
underwent surgery, confirming the presence of a
hamartomatous polyp of the gallbladder. 2
Again, to the best of our knowledge, no hamartomatous
polyp has been reported before in a
patient without a history of PJS. We believe that
this patient’s age, his personal and family history,
no clinical conditions specific to PJS, and negative
small-bowel follow-through and long-term
follow-up findings were all reassuring to exclude
the presence of underlying PJS. Another interest-
Karaosmanoglu and Blake
Figure 4. Endoscopic retrograde cholangiopancreatogram.
Postendoscopic contrast agent injection shows a dilated common
hepatic duct (arrow). The dilatation is more prominent in
the upper two-thirds of the common bile duct.
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Hamartomatous Polyp of the Gallbladder With Choledochal Cyst
1666
ing finding of this case was the presence of an
associated choledochal cyst. To the best of our
knowledge, the association of these two rare
disorders has also not been reported before.
However, it is highly speculative to hypothesize
any genetic or metabolic association between
these two abnormalities on the basis of a single
patient experience and lack of supporting literature
data.
In conclusion, although extremely rare, the
presence of a hamartomatous polyp must be
kept in mind in the pediatric age group even in
the absence of PJS. Sonography may be
extremely helpful, with its unique abilities of
high-resolution gallbladder imaging and lack of
radiation, which is especially important in the
pediatric population.
References
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J Ultrasound Med 2010; 29:1663–1666