Abstract Background Tumors of the minor papilla of the duodenum are extremely rare, and they are mostly neuroendocrine tumors, such as somatostatinomas and carcinoid tumors. However, true incidence of carcinoid tumors in minor papilla might be much higher, because patients with minor papillary tumors usually remain asymptomatic. We report a very unusual case of carcinoid tumor in a patient with complete pancreas divisum with a review of the literature. Case presentation A 56-year-old female patient was referred for evaluation of pancreatic duct dilatation noted on abdominal ultrasonography and computerized tomography. She complained of intermittent epigastric pain for 6 months. A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct. On duodenoscopy, a small, yellows, subepithelial nodule was visualized at the minor papilla; biopsy of this lesion revealed a carcinoid tumor. She underwent a pylorus-preserving pancreaticoduodenectomy. The histologic evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and metastasis to the regional lymph nodes. Conclusion Although the size of the carcinoid tumor was small and the tumor was hormonally inactive, the concomitant pancreas divisum led to an early diagnosis, the tumor had aggressive behavior. Carcinoid tumors of the minor papilla should be included in the differential diagnosis of recurrent abdominal pain or pancreatitis of unknown cause.</p

CL Cheng

E Hatzitheoklitos

E Solcia

HR Makhlouf

HY Wang

IM Modlin

J Waisberg

JD Godwin

JR Lowes

K Oberg

Kyeong Ok Kim

MC Machado

MH Kulke

MJ Malone

O Outtas

PE Stommer

RH Hawes

T Itoi

Tae Nyeun Kim

VV Singh

WM Clements

Y Noda

Yong Gil Kim

English

PubMed

Abstract Background Tumors of the minor papilla of the duodenum are extremely rare, and they are mostly neuroendocrine tumors, such as somatostatinomas and carcinoid tumors. However, true incidence of carcinoid tumors in minor papilla might be much higher, because patients with minor papillary tumors usually remain asymptomatic. We report a very unusual case of carcinoid tumor in a patient with complete pancreas divisum with a review of the literature. Case presentation A 56-year-old female patient was referred for evaluation of pancreatic duct dilatation noted on abdominal ultrasonography and computerized tomography. She complained of intermittent epigastric pain for 6 months. A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct. On duodenoscopy, a small, yellows, subepithelial nodule was visualized at the minor papilla; biopsy of this lesion revealed a carcinoid tumor. She underwent a pylorus-preserving pancreaticoduodenectomy. The histologic evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and metastasis to the regional lymph nodes. Conclusion Although the size of the carcinoid tumor was small and the tumor was hormonally inactive, the concomitant pancreas divisum led to an early diagnosis, the tumor had aggressive behavior. Carcinoid tumors of the minor papilla should be included in the differential diagnosis of recurrent abdominal pain or pancreatitis of unknown cause.</p

Kim Kyeong

Kim Tae

Kim Yong

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BMC Gastroenterology

Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain

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CASE REPORT Open Access
Carcinoid tumor of the minor papilla in complete
pancreas divisum presenting as recurrent
abdominal pain
Yong Gil Kim, Tae Nyeun Kim*, Kyeong Ok Kim
Abstract
Background: Tumors of the minor papilla of the duodenum are extremely rare, and they are mostly
neuroendocrine tumors, such as somatostatinomas and carcinoid tumors. However, true incidence of carcinoid
tumors in minor papilla might be much higher, because patients with minor papillary tumors usually remain
asymptomatic. We report a very unusual case of carcinoid tumor in a patient with complete pancreas divisum with
a review of the literature.
Case presentation: A 56-year-old female patient was referred for evaluation of pancreatic duct dilatation noted on
abdominal ultrasonography and computerized tomography. She complained of intermittent epigastric pain for 6
months. A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct. On
duodenoscopy, a small, yellows, subepithelial nodule was visualized at the minor papilla; biopsy of this lesion
revealed a carcinoid tumor. She underwent a pylorus-preserving pancreaticoduodenectomy. The histologic
evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and
metastasis to the regional lymph nodes.
Conclusion: Although the size of the carcinoid tumor was small and the tumor was hormonally inactive, the
concomitant pancreas divisum led to an early diagnosis, the tumor had aggressive behavior. Carcinoid tumors of
the minor papilla should be included in the differential diagnosis of recurrent abdominal pain or pancreatitis of
unknown cause.
Background
Tumors of the minor papilla of the duodenum are very
rare; the majority of tumors of the minor papilla of the
duodenum are neuroendocrine tumors (NETs), such as
somatostatinomas and carcinoid tumors [1-7]. Carcinoid
tumors arise from enterochromaffin cells, and gastroin-
testinal carcinoids are usually located in the appendix,
ileum, stomach, and rectum. Carcinoids of the minor
papilla are extremely rare; only 8 cases have been
reported in the literature [1-8].
Pancreas divisum is the most common congenital
anomaly of the pancreas, resulting from failure of fusion
between the dorsal and ventral pancreatic ducts. Most
exocrine secretions of the pancreas drain through the
dorsal pancreatic duct and the minor papilla in the
pancreas divisum. Patients with pancreas divisum
usually have no clinical symptoms, but sometimes have
recurrent abdominal pain or pancreatitis when distur-
bances in drainage of pancreatic secretion through the
minor papilla occur.
We report a very unusual case of a carcinoid tumor of
the minor papilla arising in a patient with complete pan-
creas divisum who presented with recurrent abdominal
pain.
Case persentation
A 56-year-old female was referred to our department for
evaluation of dilatation of the main pancreatic duct
noted on abdominal ultrasonography and CT scan. She
complained of intermittent epigastric pain 6 months
prior to the referral. She did not drink alcohol or take
any medications, and had no significant medical history.
She denied sweating, diarrhea, or facial flushing.
* Correspondence: tnkim@ynu.ac.kr
Department of Internal Medicine, Yeungnam University College of Medicine,
Daegu, South Korea
Kim et al. BMC Gastroenterology 2010, 10:17
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© 2010 Kim et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons
Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly cited.
Laboratory data on admission were as follows: total
bilirubin, 0.47 mg/dL; AST, 41 U/L; ALT, 33 U/L; ALP,
161 U/L; GGT, 32 U/L; and amylase, 147 U/L. The
complete blood count, urine analysis, and serum electro-
lytes were within normal limits. The CA 19-9 was 7.99
U/ml and the 5-HIAA was 21.44 mg/day.
On the CT scan, the pancreatic duct was diffusely
dilated without a definite mass and the common bile
duct was slightly dilated (Figure 1). Magnetic resonance
cholagiopancreatography (MRCP) showed a dilated dor-
sal pancreatic duct crossing over the common bile duct
with stenosis at the minor papailla. The ventral duct
appeared to be normal and there was no communica-
tion between the dorsal and ventral ducts, indicating
pancreas divisum (Figure 2). The common bile duct was
slightly dilated, and narrowing of the distal common
bile duct (CBD) was noted without passage disturbance.
A duodenoscopic examination revealed a 1 cm yellow,
bulging, nodular lesion with an intact mucosal surface
in the minor papilla. The orifice of the minor papilla
was not visible due to the nodule and cannulation was
not possible (Figure 3A). On endoscopic retrograde cho-
langiopancreatography (ERCP), the major papilla
appeared to be normal. Injection of contrast through
the major papilla revealed a short and slender ventral
duct confined to the pancreas head with no visualization
of ducts in the body and tail. The ventral duct gradually
diminished in caliber and was arborizing (Figure 3B).
Endoscopic ultrasonography (EUS) showed a 1.3 cm ill-
defined hypoechoic mass in the submucosal layer of the
minor papilla (Figure 4). Multiple deep biopsies of this
nodule revealed a carcinoid tumor.
Under the diagnosis of a carcinoid tumor of the minor
papilla in complete pancreas divisum, the patient under-
went a pylorus-preserving pancreaticoduodenectomy.
The resected specimen consisted of duodenum, pan-
creas, and CBD. There was a 1.2 × 1.0 cm submucosal
lesion of the minor papilla (Figure 5).
On the microscopic examination, uniform and round-
shaped cells were visualized in or around the heteroto-
pic pancreas in the minor papilla, arranged in a solid
nest or tubular pattern, suggesting a carcinoid tumor.
Figure 1 Abdominal CT finding. The pancreatic duct was diffusely dilated without a definite obstructive mass lesion.
Kim et al. BMC Gastroenterology 2010, 10:17
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Figure 2 Magnetic resonance cholangiopancreatographic finding. The dorsal pancreatic duct was slightly enlarged with stenosis at the
minor papilla. Furthermore, it crossed over the common bile duct. The ventral duct appeared to be normal and there was no communication
between the dorsal and ventral ducts, indicating pancreas divisum.
Figure 3 ERCP finding. A, Duodenoscopic examination revealed 1 cm sized yellowish bulging nodular lesion with intact mucosal surface at
minor papilla. B, Injection of contrast through major papilla revealed short and slender ventral duct confined to pancreas head with no
visualization of ducts in body and tail. The ventral duct was gradually diminishing in caliber and arborizing.
Kim et al. BMC Gastroenterology 2010, 10:17
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Figure 4 Endoscopic ultrasonography finding. There was 1.3 cm sized ill defined hypoechoic mass in submucosal layer at minor papilla.
Figure 5 Gross findings of resected specimen. The resected specimen was composed of duodenum, pancreatic head with a part of
pancreatic body and common bile duct. There was 1.2 × 1.0 cm sized submucosal lesion at minor papilla (arrow). There was no communication
between common bile duct (CBD) and pancreatic duct (PD). Furthermore, dorsal pancreatic duct only connected with duct of Santorini and
without any branch to major papilla. These findings were appropriate to pancreas divisum.
Kim et al. BMC Gastroenterology 2010, 10:17
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There was vascular invasion, but no perineural invasion
was noted (Figures 6A, B). Among the four resected
regional lymph nodes, 2 metastatic nodes were detected.
The tumor cells showed immunoreactivity to neuroen-
docrine markers, such as synaptophysin and chromogra-
nin (Figures 6C, D) The patient has been without
abdominal pain for 1 year after surgery.
Discussion
The term carcinoid has been replaced with NET based
on the development of diagnostic tools and immuno-
chemistry. Recently, NETs have been reclassified within
the spectrum of gastroenteropancreatic-neuroendocrine
tumours (GEP-NETs) [9]. However, the term “classical
carcinoid” is used synonymously with the term seroto-
nin-producing GEP-NETs [10]. A classical carcinoid
tumor is usually still referred to as a carcinoid tumor.
The incidence of gastrointestinal carcinoids is 1.6-2.0
cases per 100, 000 persons per year [11], but the true
prevalence may be higher [12]
Although more than 70 cases involving carcinoid
tumors of the major papilla have been reported [13],
only 8 cases of carcinoid tumors involving the minor
papilla have been reported [1-8]. Carcinoids and endo-
crine cell micronests (ECMs) in the minor papilla occur
more frequently than generally thought. In a single sur-
gical specimens and autopsy study, the incidence of car-
cinoids and neoplastic ECMs of the minor papilla could
reach 10%. Furthermore, carcinoids in the minor papilla
are twice as common as carcinoids of the major papilla,
and neoplastic ECMs of the minor papilla are found five
times as often [14]. This discrepancy may be explained
by the fact that tumors of the major papilla are more
likely to develop symptoms, such as jaundice or
Figure 6 Microscopic examination. A, B, Heterotopic pancreas in the duodenal submucosa and muscularis propria around the minor papilla
was noted. There were uniform and round-shaped cells in or around the heterotopic pancreas and they were arranged in a solid nest or
tubular pattern. These findings were suggestive of a carcinoid tumor. There was vascular invasion, but no perineural invasion was noted. (H&E
stain A: ×10, B: ×100) C, D, According to immunohistochemical staining, the tumor cells were immunoreactive with neuroendocrine markers,
such as synaptophysin and chromogranin.(C:Synaptophysin stain ×100; D: Chromogranin stain ×100)
Kim et al. BMC Gastroenterology 2010, 10:17
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Page 5 of 7
abdominal pain, due to ampullary obstruction, whereas
patients with minor papillary tumors usually remain
asymptomatic because there is no biliary or pancreatic
obstruction.
Carcinoid tumors of the minor papilla of the duode-
num are very difficult to diagnose because they are
usually small in size and located at the submucosal area,
and they are frequently asymptomatic [15] and rarely
accompanied with endocrine manifestations [13]. In our
case, although the size of the carcinoid tumor was small
and the tumor was hormonally inactive, the concomitant
pancreas divisum led to an early diagnosis because
obstruction of the minor papilla due to the carcinoid
tumor caused recurrent abdominal pain and resulted in
dilatation of the main pancreatic duct. Of the eight
reported cases of carcinoid tumors involving the minor
papilla in the literature, five cases have been associated
with pancreas divisum and presented with recurrent
abdominal pain or pancreatitis [3-7].
The endoscopic features of carcinoid tumors are small,
round, sessile, or polypoid lesions with a smooth sur-
face. Carcinoid tumors usually have normal overlying
mucosa and seldom ulcerate [16]. An EUS may be use-
ful for the diagnosis of carcinoids and evaluation of
regional lymph node metastasis, but CT or MRI scans
are not helpful in most cases because of the small size.
Carcinoid tumors usually appear as a homogenous, well-
demarcated, and mildly hypoechoic or isoechoic mass
that arises from the second layer on EUS. In addition,
EUS can define the size, tumor invasion, and regional
lymph node metastasis [16]. The pre-operative diagnosis
of carcinoid tumors of the duodenal papillae is largely
dependent upon endoscopic biopsy. However, a correct
diagnosis is made in only 14% of the patients13 because
the tumors are usually submucosal in location [13]. As
in our case, multiple deep biopsies are required to
enhance the tissue diagnosis.
Though endoscopic resection may be an alternative to
surgical resection [8,17], radical surgery is usually
recommended as the treatment of ampullary carcinoid
tumors [13,18] because carcinoid tumors of this area
appear to have more aggressive biology with presence of
metastasis in approximately 50% of the cases, irrespec-
tive of the size of the primary tumor or mitotic activity
[13,19,20]. In our case, despite the tumor being 1 cm in
diameter, the histologic examination revealed duodenal
and vascular invasion and metastasis to the regional
lymph nodes. In conclusion, carcinoid tumors of the
minor papilla are very rarely found, but the true inci-
dence might be much higher, and the tumors frequently
have aggressive behavior. Carcinoid tumors of the minor
papilla should be included in the differential diagnosis
of recurrent abdominal pain or pancreatitis of unknown
cause.
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
Authors’ contributions
All authors read and approved this manuscript. YGK analyzed and
interpreted the patient’s data. KOK collected the patient’s data. TNK
supervised this case report and followed up the patient.
Competing interests
The authors declare that they have no competing interests.
Received: 15 November 2009
Accepted: 12 February 2010 Published: 12 February 2010
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Cite this article as: Kim et al.: Carcinoid tumor of the minor papilla in
complete pancreas divisum presenting as recurrent abdominal pain.
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A 5-decade analysis of 13,715 carcinoid tumors. Cancer

Ajioka Y: Carcinoids and endocrine cell micronests of the minor and major duodenal papillae. Their incidence and characteristics. Cancer

Carcinoid of the minor papilla in incomplete pancreas divisum presenting as acute relapsing pancreatitis. Pancreas

Carcinoid tumors. An analysis of 2,837 cases.

Cotton PB: Obstructive pancreatitis: unusual causes of chronic pancreatitis.

dos Santos HV, Fernezlian SM, Capelozzi VL: Carcinoid of the minor duodenal papilla associated with pancreas divisum: Case report and review of the literature. Clinics

Early somatostatinoma of the papilla of the duct of Santorini. Arch Surg

ED: Histological typing of endocrine tumours.

GA: Endoscopic snare papillectomy for tumors of the duodenal papillae. Gastrointest Endosc

Guidelines for the management of gastroenteropancreatic neuroendocrine tumours (including bronchopulmonary and thymic neoplasms). Part I-general overview. Acta Oncol

HG: Carcinoid of the ampulla of Vater. Clinical characteristics and morphologic features. Cancer

Moriyasu F: Endoscopic resection of carcinoid of the minor duodenal papilla.

Pinotti HW: [Carcinoid tumor of the duodenal papilla]. Rev Hosp Clin Fac Med Sao Paulo

RJ: Carcinoid tumors.

S: [Pancreatic panniculitis with intraductal carcinoid tumor of the pancreas divisum]. Ann Dermatol Venereol

Seifert E: Somatostatinoma of Vater’s papilla and of the minor papilla. Cancer

YJ: Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera.

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Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain

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