310 J La state Med soc VoL 162 november/December 2010
Journal of the Louisiana State Medical Society
clinical case oF the month
Maneesh K. Gupta, MD; Jordan J. Karlitz, MD; Daniel L. Raines, MD;
Sander S. florman, MD; and fred A. Lopez, MD (Section Editor)
Heterotopic pancreas (HP) is typically an asymptomatic
malformation that can present anywhere along the
gastrointestinal tract. It is often found incidentally as a
single focal mass in patients with abdominal complaints
undergoing routine diagnostic testing. HP occasionally
presents symptomatically, and the manifestations vary
depending on the location of the lesion. We describe the first
reported case of symptomatic jejunal HP which presents as
a circumferential infiltrative lesion with multiple foci rather
than a single focal mass. This is accompanied by a review
of the current literature regarding the pathogenesis, clinical
manifestations, and treatment of HP.
A 37-year-old woman with a history of systemic lupus
erythematosus and cadaveric kidney transplant in 1997 was
referred to our institution for recurrent nausea, vomiting,
diarrhea, and abdominal pain of two years duration. Her
symptoms occurred in discrete episodes requiring repeated
hospitalizations for dehydration and abdominal pain. On
physical exam, her abdomen was tender to palpation in
the left upper quadrant and epigastrum, with voluntary
guarding but no rebound tenderness.
Extensive evaluation including laboratory studies,
abdominal ultrasound, upper endoscopy, and colonoscopy
were unrevealing. An abdominal computed tomographic
(CT) scan with oral and intravenous contrast demonstrated
an area of abnormal small bowel localized to the
proximal jejunum (Figure 1). Capsule endoscopy was
subsequently performed to evaluate the small bowel and
was unremarkable. Due to persistent symptoms, the patient
underwent single bowel enteroscopy (SBE) to visualize
directly and to biopsy the abnormal jejunal segment seen
Examination by SBE revealed a 6cm grossly edematous
and circumferentially ulcerated segment of mucosa located
approximately 100cm distal to the Ligament of Treitz
(Figure 2). The lesion was biopsied; however, pathological
examination only revealed mild vascular congestion.
The patient was eventually taken for surgical resection.
Intraoperatively, the lesion was visualized. In addition,
a separate ulcerated lesion was seen in the small bowel
10cm proximal to the original lesion. Both lesions were
resected, and histological examination revealed heterotopic
pancreatic tissue involving the adventitia, muscularis
propria, and submucosa extending to the muscularis
mucosa (Figure 3). There was no evidence of vasculitis.
The patient experienced symptoms of abdominal pain
and nausea postoperatively but these symptoms resolved
completely within one month.
figure 1. Axial computed tomographic image of the abdomen
demonstrating gross thickening of a jejunal segment (arrows).
J La state Med soc VoL 162 november/December 2010 311
Heterotopic pancreas (HP) is defined as pancreatic
tissue that lacks an anatomic or vascular communication
with the normal body of the pancreas.1-3 Although HP can
occur throughout the entire gastrointestinal tract, it is most
commonly found in the stomach (25%-38%), duodenum
(17%-36%), and jejunum (15%-21%).4 Rare cases have
described HP in the esophagus, biliary tract, gallbladder,
spleen, and mesentery. HP can be pathologically subdivided
into four subtypes. Type 1 heterotopia consists of typical
pancreatic tissue. The remaining subtypes consist of specific
components of normal pancreatic tissue such as pancreatic
ducts only (type II), acinar tissue only (type III), or islet cells
only (type IV).5 Heterotopic pancreatic tissue can be found in
patients of any age and is slightly more common in men.4
The development of heterotopic pancreatic tissue
is unclear. The two predominant theories of origin of
HP involve misplacement of pancreatic tissue during
development versus tissue metaplasia. Embryologically, the
pancreas develops from invaginations of endodermal tissue
in the primitive duodenum. The ventral aspect forms the
head of the pancreas and the dorsal aspect forms the body
and tail. The misplacement theory proposes that, during
rotation of the foregut, several elements of the primitive
pancreas become separated and eventually form mature
pancreatic tissue along the length of the gastrointestinal
tract.2 The metaplasia theory states that pancreatic
heterotopia arises from areas of pancreatic metaplasia of
the endoderm which migrate to the submucosa during
Heterotopic pancreatic tissue is a common incidental
finding in patients undergoing laparotomy for other
indications or at autopsy. The reported frequency of this
finding during laparotomy is 0.5% and at autopsy is 1.7%.7
Despite the relatively frequent occurrence of HP, the vast
majority of these cases are asymptomatic.8 When present,
symptoms vary depending on the anatomical location and
size of the lesion. Abdominal pain, nausea, vomiting, and
gastrointestinal bleeding are the most commonly reported
symptoms and are most likely to be seen with lesions greater
than 1.5cm in diameter.2 Pain associated with HP may be
related to the local secretion of hormones and enzymes
resulting in tissue inflammation or chemical irritation.4
Pain may also be related to mechanical obstruction of
the intestinal lumen, especially when associated with
nausea or vomiting. Gastric lesions are the most likely to
be symptomatic, presenting with either epigastic pain or
symptoms of gastric outlet obstruction due to a pre-pyloric
mass.9 Rarely, jejunal lesions may result in intestinal
obstruction or intussusception.10-12 Even after finding an HP
lesion in a patient with the above mentioned symptoms,
other causes must be ruled out before the symptoms can
be attributed to the HP.8,10,13,14
HP also may present with symptoms related to
complications similar to those normally associated with
the pancreas. Cases of pancreatitis due to gastric lesions
have been reported.15,16 Pseudocyst formation complicating
duodenal lesions is another known complication.17,18
Malignant transformation of heterotopic pancreas can
rarely occur with up to 15 cases reported.19,20 In order to be
identified as malignancy derived from HP, the malignant
figure 2. Endoscopic image of the lumen of the proximal jejunum
seen on single balloon enteroscopy. The mucosa in this segment is
circumferentially edematous. The segment was marked with india
ink in preparation for surgical resection.
figure 3. Microscopic pathology of the resected segment of intes-
tine. Heterotopic pancreatic tissue is seen involving the adventitia,
muscularis propria and submucosa (arrow).
312 J La state Med soc VoL 162 november/December 2010
Journal of the Louisiana State Medical Society
tissue must be within or in close proximity to the heterotopic
focus. Additionally, a transition between the carcinoma and
normal pancreatic structures must exist. Fully developed
ducts and acini in the non-neoplastic heterotopic pancreatic
tissue must also be seen. Overall, adenocarcinoma arising
from HP has a better prognosis than malignancy which
arises from the normally located pancreas.8
HP has several characteristic radiographic and
endoscopic features that aid in its identification. Gastric
HP classically presents as a rounded filling defect with a
central indentation on barium studies or as a broad based,
umbilicated, submucosal lesion on endoscopy.8 This central
umbilication results from a subjacent pancreatic duct.21
However, these features are not always present, leading
to a difficulty in diagnosis. In 1999, Hsia et al reported
that only 3 of 17 cases of HP presented preoperatively
with the endoscopic finding of a submucosal lesion with a
central indentation.22 Various imaging tests can be used to
identify heterotopic pancreas in the gastrointestinal tract.
It is important to stress that these tests are almost always
performed to evaluate more common causes of abdominal
symptoms, and the finding of HP is usually incidental.
Computed tomographic findings are usually nonspecific
although heterotopic pancreatic tissue can enhance to the
same degree as normal pancreas with intravenous contrast.
Newer technologies such as capsule endoscopy and single
bowel enteroscopy are being used to detect lesions in the
small bowel which were previously inaccessible without
surgery.8 Even when lesions are identified endoscopically,
biopsies are often too superficial to reveal the submucosal
lesions, and show only chronic inflammation, as in this
case.23 Endoscopic ultrasound (EUS) is the standard test
to evaluate submucosal lesions of the gastrointestinal
tract. In the gastric antrum, EUS can diagnose submucosal
heterotopic pancreas (pancreatic rests) ranging from 0.5-
2cm in diameter, and when combined with fine needle
aspiration the cytological evaluation has a sensitivity of
The management of HP is a controversial topic. Surgical
resection of HP should be performed in symptomatic
patients after more common causes of abdominal
complaints such as peptic ulcer disease, gastro-esophageal
reflux disease, and biliary disease have been ruled out.
Benign asymptomatic lesions generally do not require
surgical intervention. Ormarsson et al followed 32 patients
with HP of the stomach or small bowel for 13 years and
found that there was no malignant transformation in any
of the patients over this time. The literature is divided on
the management of asymptomatic and incidental lesions.
Some state that management should include frequent
endoscopy, while others suggest that there is no need for
increased surveillance. In lesions with malignant changes
or in which malignancy is uncertain, surgical resection is
Although heterotopic pancreas is not uncommon, this
is the first reported case of jejunal HP that presents as a
circumferential mural infiltration with multiple foci rather
than a single focal mass. This patient had two separate
jejunal lesions which involved the adventitia, muscularis
propria, and submucosa resulting in symptoms of recurrent
intestinal obstruction. Additionally, this case highlights
the difficulty in making a diagnosis of HP even with the
aid of newer techniques such as capsule endoscopy and
SBE. Although an area of gross inflammation was located
endoscopically in the jejunum, a diagnosis of HP could not
be made until the specimen was surgically excised.
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J La state Med soc VoL 162 november/December 2010 313
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Hsu SD, Chan DC, Hsieh HF, et al. Ectopic pancreas presenting as
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Chak A, Canto MI, Rosch T, et al. Endosonographic differentiation
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Dr. Gupta is a first-year internal medicine resident at Louisiana State
University Health Sciences Center, new orleans. Dr. karlitz is an
assistant professor of clinical medicine in the Department of Medicine,
Section of Gastroenterology at Tulane University Medical Center, new
orleans. Dr. Raines is an assistant professor of clinical medicine in the
Department of Medicine, Section of Gastroenterology at Louisiana
State University Health Sciences Center, new orleans. Dr. florman
is a professor of surgery at Mount Sinai Medical Center and director
of the Recanati/Miller Transplant institute in new York. Dr. Lopez is
the Richard Vial Professor of medical education at Louisiana State
University Health Sciences Center in new orleans.
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