Dig Surg 2006;23:262–264
DOI: 10.1159/000096158
Ectopic Pancreas in the Ampulla of Vater with
Obstructive Jaundice
A Case Report and Review of Literature
Shao-Jiun Chou a, b, Yu-Wei Chou a, Hsiang-Chun Jan a, b,
Victor T.K. Chen a, b, Tzu-Hung Chen a, b
a
Division of General Surgery, Department of Surgery, Cardinal
Tien Hospital, College of Medicine, Fu Jen Catholic University,
and b Tri-Service General Hospital and National Defense Medical
Center, Taipei, Taiwan/ROC
Key Words
Ectopic pancreas Ampulla of Vater Periampullary tumor Total ampullectomy Sphincterotomy
Abstract
Ectopic pancreas is an uncommon condition and is usually found
in the gastrointestinal tract, such as stomach, duodenum and jejunum. However, ectopic pancreas in the ampulla of Vater is rare and
its clinical presentations may be similar to periampullary cancer. It is
difficult to diagnose preoperatively. We present such a case where
the diagnosis was proven postoperatively. Our patient, a 51-year-old
man, presented with epigastric pain, jaundice, weight loss and abnormal laboratory data. Imaging study, including abdominal sonography, abdominal computerized tomography with contrast and endoscopic retrograde cholangiopancreatography, showed a mass
protruding into the ampulla of Vater. The mass was resected and histological examination revealed an ectopic pancreas. The patient presented with symptoms of periampullary tumor but the imaging
study did not reveal an obvious lesion for us consider the possibility
of ectopic pancreas. Surgical excision is indicated for symptomatic
Copyright © 2006 S. Karger AG, Basel
cases.
1
2
Introduction
Ectopic pancreas is defined as pancreatic tissue that lacks direct or vascular connection to normal pancreas [1]. Ectopic pancreas is an uncommon condition. In 70% of the cases it is found
in the stomach, duodenum and jejunum [2]. We present a case of
ectopic pancreas in the ampulla of Vater (AoV). Only several such
cases have been reported in the literature [3–9]. The common
presenting symptoms include epigastric pain and jaundice [2].
Our case also had weight loss and abnormal laboratory data,
which were not seen in previous reports.
Case Report
A 51-year-old man presented with epigastric pain for almost
1 year. The pain was dull, intermittent and non-referring. He had
a weight loss of 5 kg within 4 months. He consulted a local hospital and was treated for gastritis. Physical examination on admission showed epigastric tenderness, gross jaundice and recent
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Fig. 1. Marked dilatation of intrahepatic biliary ducts (arrow).
Fig. 2. The CBD was dilated in the ampullary region (arrow) and
the pancreas was swollen.
weight loss. Laboratory data included normal hematocrit and
WBC, but elevated serum amylase 806 U/l (normal 20–120 U/l),
lipase 1,229 U/l (normal 8–57 U/l), -GT 751 U/l (normal 7–
50 U/l), ALT 195 U/l (normal 10–60 U/l), APT 103 U/l (normal
5–40 U/l), total bilirubin 10.6 mg/dl (normal 0.4–2 mg/dl), direct
bilirubin 8.54 mg/dl (normal 0–0.4 mg/dl), and LDH 291 U/l
(normal 98–192 U/l). Tumor markers of CA 19-9 was 209.69 U/l
(normal 0–37 U/l), CEA was 4.93 ng/ml (normal !5 ng/ml), and
AFP was 1.7 ng/ml (normal ! 20 ng/ml). Chest and abdomen Xrays were unremarkable. Abdominal sonography showed a dilated common bile duct (2.0 cm) and dilated intrahepatic ducts. Abdominal computerized tomography (CT) revealed a dilated com-
Case Reports
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5
4
6
Fig. 3. ERCP showed a protruding mass at the AoV.
Fig. 4. Distal CBD showed marked narrowing with near total ob-
Fig. 5. AoV showed gross hypertrophy without the mass.
Fig. 6. Histopathological sections showed pancreatic acini and
struction.
ducts.
mon bile duct extending down to the ampullary region (fig. 1,
2). Endoscopic retrograde cholangiopancreatography (ERCP)
showed a dilated common bile duct and a mass protruding into
the AoV (fig. 3, 4). A biopsy was done and revealed chronic inflammation. The Whipple procedure was performed due to the
suspicion of tumor of AoV. The resected specimen of ampulla
showed gross hypertrophy without the mass (fig. 5). Histological
evaluation revealed a mixture of pancreatic acini and ducts
(fig. 6). The postoperative course was smooth and the patient was
discharged on postoperative day 10. During the postoperative follow-up his symptoms were relieved and laboratory data returned
to a normal range.
Case Reports
Discussion
The diagnosis of ectopic pancreas is easy if it shows the typical
landmark of ‘central dimpling’. However, in our case, such a characteristic sign was not seen. The ectopic pancreatic tissue may be
too small and difficult to see near the orifice of the AoV.
Ectopic pancreas is usually asymptomatic, but it is capable of
producing clinical symptoms, depending on its location and size
[10]. In reviewed reports, the most common sites include stomach, duodenum, and jejunum, which account for about 70% of
cases [2]. Only a few cases have been reported in the AoV. The
sizes reported range from 2.0 to 25 mm [3–9]. The most common
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presenting symptoms were abdominal pain (45.5%) and epigastric discomfort (12.0%). In our case, ectopic pancreas located in
AoV presented with epigastric pain, gross jaundice and weight
loss. The symptoms of ectopic pancreas in the AoV are similar to
those of periampullary cancer. It is very difficult to differentiate
them based on clinical symptoms alone.
Useful imaging study of the periampullary region includes abdomen sonography, endoscopic ultrasonography (EUS), abdomen CT, magnetic resonance imaging (MRI), and ERCP. Abdominal sonography may not provide much information in such patients, as in our case, because the ectopic pancreatic tissue is small
and located in the submucosal layer of AoV [11]. In EUS the ectopic pancreas in the gastrointestinal tract appears hypoechoic or
has mixed echogenic lesions in the submucosa [12]. EUS features
of ectopic pancreas in AoV have not been reported. Abdominal
CT can evaluate the internal organs near to the periampullary
region and rule out any periampullary abnormality. ERCP with
biopsy is the most important tool in such cases. Ectopic pancreatic tissue may show the ‘central dimpling’ in ERCP if the tissue
is large enough. Endoscopic sphincterotomy may supply larger
specimens for diagnosis, however it has more complications, such
as hemorrhage, perforation, or pancreatitis.
Hayes-Jordan et al. [13] pointed out ectopic pancreas is associated with muscle hypertrophy. Gaspar Fuentes et al. [14] divided
ectopic pancreas into four types: type I composed of all cell types
(total heterotopia); type II composed of ducts only (canalicular
heterotopia); type III composed of acinar cells only (exocrine heterotopia), and type IV composed of islets of Langerhans only (endocrine heterotopia). However, in our case, histological findings
revealed a mixture of acini and ducts.
The management of ectopic pancreas depends on the presence
of symptoms or not. If the patient is asymptomatic, conservative
treatment with regular follow-up is recommended. If the patient
is symptomatic, as our case, surgical management is indicated.
The size and location of ectopic pancreatic tissue determine what
kind of procedure should be performed. Tanaka et al. [15] recommended resection of the pancreatic tissue-bearing area, while Jochimen et al. [16] recommended surgical excision of ectopic pancreas if symptoms persisted after supportive treatment failed. If
we can localize the ectopic pancreatic tissue preoperatively by imaging study, limited excision is needed. If ectopic pancreatic tissue
is not well circumstanced, wide excision is recommended, such as
total ampullectomy. However, wide excision may be technically
difficult. We suggest biopsy and sphincterotomy to identify the
anatomy and histology first, and then excise the area as much as
possible without damaging the common bile duct and pancreatic
duct.
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Tzu-Hung Chen, MD
Division of General Surgery, Department of Surgery
Cardinal Tien Hospital
362, Chung Cheng Rd.
Hsintien Taipei Hsien 23137 (Taiwan/ROC)
Tel. +886 2 2219 3391 66711, Fax +886 2 8665 9727
E-Mail surgery6711@sina.com.tw
Acknowledgement
We thank Miin-Fu Chen, MD, President of the Taiwan Surgical Association, for his help in revising the manuscript.
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Case Reports