Abdulhalem Alzein

Abduljalil Benhamida

Ahmed Alzaraa

Husam Mousa

Keel

M Brugge

MO Ustun

N Jani

Neha Dalal

R Salvia

S Blandamura

v Friebe

Valeri Udom

World Journal of Surgical Oncology

English

PubMed

Crossref

Combined endocrine and exocrine tumours of the pancreas

Springer - Publisher Connector

BioMed Central
World Journal of Surgical Oncology
ssOpen AcceCase report
Combined endocrine and exocrine tumours of the pancreas
Ahmed Alzaraa*1, Valeri Udom2, Husam Mousa1, Abdulhalem Alzein1, 
Abduljalil Benhamida1 and Neha Dalal3
Address: 1Department of General Surgery, Tameside General Hospital, Manchester, UK, 2Department of Radiology, Tameside General Hospital, 
Manchester, UK and 3Department of Histopathology, Tameside General Hospital, Manchester, UK
Email: Ahmed Alzaraa* - ahmedwahabf@gmail.com; Valeri Udom - vudom52@aol.com; Husam Mousa - hmousa2006@hotmail.co.uk; 
Abdulhalem Alzein - aelzein73@yahoo.com; Abduljalil Benhamida - abduljalil.benhameda@tgh.nhs.uk; Neha Dalal - neha.dalal@tgh.nhs.uk
* Corresponding author    
Abstract
Background: Cystic neoplasms of the pancreas comprise 10%–15% of pancreatic cystic lesions,
with the serous cystadenoms being the commonest. The association of exocrine and endocrine
tumours of the pancreas unrelated to Von Hipple Lindau disease is very rare. Very few cases have
been reported in the literature. We present another case of both these tumours in one patient.
Case presentation: A female patient was seen in the surgical clinic for a pain in the right groin.
Clinical examination and investigations confirmed a diagnosis of combined endocrine and exocrine
tumours of the pancreas. She underwent surgery and is under regular follow-up in the surgical
clinic.
Conclusion: Biphasic differentiation of pancreatic stem cell during embryological development
could happen and may result in combined endocrine and exocrine tumours of the pancreas. Imaging
studies are excellent in diagnosing theses lesions. Surgery has a central role and could be curative.
Background
The coexistence of endocrine and exocrine tumours of the
pancreas is very rare. To our knowledge, only seven simi-
lar cases have been reported in the literature [1-6]. Here,
we present the 8th case with the radiological and histolog-
ical findings.
Case presentation
A 73 years-old lady was seen in the clinic in August 2006
for a right inguinal pain which had been present for a few
months. She had no significant past medical history.
Abdominal examination revealed a mass in the left upper
quadrant. There were no other physical findings.
Computed tomography (CT) of the abdomen confirmed
a 12.3 cm × 8.2 cm mass arising from the body and the tail
of the pancreas and extending into the splenic hilum (Fig-
ure 1). There was no lymphadenopathy involving the
porta hepatis or the retroperitoneum. There were large
simple cysts in the liver and the kidney. There were no
other features of disseminated malignancy seen. Gastric
endoscopy revealed an external mass pressing on the
greater curvature of the stomach. CT chest was normal.
The patient underwent tumour excision together with dis-
tal pancreatectomy and splenectomy in December 2006.
Histopathology report macroscopically showed a cystic
mass measuring 13 × 10 × 10 cm and weighed 518 gm.
The cut surface was spongy and multinodular. Microscop-
Published: 14 September 2007
World Journal of Surgical Oncology 2007, 5:103 doi:10.1186/1477-7819-5-103
Received: 4 May 2007
Accepted: 14 September 2007
This article is available from: http://www.wjso.com/content/5/1/103
© 2007 Alzaraa 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.Page 1 of 4
(page number not for citation purposes)
World Journal of Surgical Oncology 2007, 5:103 http://www.wjso.com/content/5/1/103ically, the tumour was composed of multiple cystic spaces
lined by low columnar-cuboidal serous type epithelium
with fibrocollagenous walls (figure 2). There was a focal
papillary pattern. No significant cytological atypia or
mitosis was seen. Within the stroma, there were nests of
cords and trabeculae of small monotonous cells with
speckled chromatin (figure 3). Immunohistochemically,
these cells were positive for neuroendocrine markers; syn-
aptophysin, chromogranin and neuron specific enolase.
The appearance, therefore, represent a typical microcystic
serous cystadenoma with an associated neuroendocrine
tumour, probably of pancreatic origin and of uncertain
malignant potential. The spleen was removed at the same
time and there was extensive infiltration of the fibrocolla-
genous tissue at the splenic hilum by the neuroendocrine
tumour.
The patient has not shown any sings of recurrence and is
under regular follow-up in the surgical clinic.
Discussion
Cystic neplasms comprise 10%–15% of pancreatic exo-
crine lesions [2]. They include a spectrum of benign,
malignant and borderline neoplasms, of which, serous
cystadenomas are most frequent. Pancreatic Endocrine
Tumours (PETs) are rare (1 per 100,000 population),
thought to originate from immature stem cells that are
part of the neuroendocrine system. They are classified into
functioning (70% of cases) if responsible for a clinical
syndrome related to hormonal release, or non-function-
ing (15–30% of cases) if there are no hormone related
symptoms [7] and may cause obstructive symptoms of the
biliary tract, duodenum, gastrointestinal bleeding or
abdominal mass. The commonest PETs are insulinomas
and gastrinomas. The correlation between the morpho-
logical features and the clinical behaviour of PET is poor,
and only the existence of metastatic disease indicates
malignancy [5,6]. Virtually always PETs are treated surgi-
cally as they have significant malignant potential [5], and
Cystic spaces on the left lined by serous type epithelium with a small sheet of endocrine tumor composed of smal  niformcells wit  round uclei (×20)Figure 3
Cystic spaces on the left lined by serous type epithelium with 
a small sheet of endocrine tumor composed of small uniform 
cells with round nuclei (×20).
CT abdomen showing the mass arising from the body and the tail of the pancreas and xtending into the spl nic hilumFigure 1
CT abdomen showing the mass arising from the body and the 
tail of the pancreas and extending into the splenic hilum.
A low power view of the cystic spaces lined by cuboidal ser us epithelium representing exo rine tumor with a cribri-fo m h e  of endocrin  tumor on the Right (×10)Figure 2
A low power view of the cystic spaces lined by cuboidal 
serous epithelium representing exocrine tumor with a cribri-
form sheet of endocrine tumor on the Right (×10).Page 2 of 4
(page number not for citation purposes)
World Journal of Surgical Oncology 2007, 5:103 http://www.wjso.com/content/5/1/103if the symptoms are not controlled, chemotherapy may be
tried but response is limited. The coexistence of pancreatic
exocrine and endocrine tumours is very rare. The first case
was described by Keel et al in 1996 [6]. Embryologically,
endocrine and exocrine pancreas develop from the endo-
dermal lining of the duodenum, therefore, the potential
of pancreatic ductal stem cells for biphasic differentiation
along either glandular epithelial or neuroendocrine path-
ways is not unexpected [5,6]. Indeed, biphasic differentia-
tion is observed in many different pancreatic disorders,
both benign and malignant, and can happen in two pat-
terns. The first pattern of mixed differentiation produces
tumours showing a combination of PET-like features and
features of either ductal or acinar cell. The second pattern
is manifested by the concurrent development of distinct
epithelial and neuroendocrine cells within the same
tumour [5].
Cystic neoplasms are a heterogeneous group of tumours
with a real problem regarding differential diagnosis
between neoplastic and inflammatory lesions owing to
the similarities in the clinical presentations and in the
characteristics visualized in imaging studies [1,3,4]. The
WHO classification of exocrine tumours of the pancreas is
based on the histopathological features of the epithelial
wall, which are the main factor in the differential diagno-
sis with cystic lesions of the pancreas [4].
Women in their 50s seem to be the population most
affected by both endocrine and exocrine tumours and any
portion of the pancreas can be involved, but they are most
frequently detected in the pancreatic head [4,7]. The asso-
ciation of endocrine pancreatic tumour with cystadeno-
mas is already known in patients with Von Hipple Lindau
(VHL) disease, but the same association unrelated to VHL
is quite rare, but not exceptional. In VHL, the pancreas is
involved in up to 77% of patients, 12.3% serous cystade-
nomas, 12.3% by endocrine tumours and in 11.5% by
combined lesions [1].
Clinically, cystic neoplasms are slow growing with no rel-
evant symptoms or signs. When clinical sings are present,
they may help pinpoint the organ of origin but never the
type of pathology. Abdominal discomfort is the most
common symptom. Weight loss, palpable mass, jaundice
and obstruction of the upper gastrointestinal tract are rare
and may be correlated to an extensive growth of the lesion
[4]. More than 1/3 of these lesions are incidentally
detected by abdominal ultrasound or CT scan performed
for the evaluation of other condition [1,3,4]. Very little
information is available on the growth rate of serous cys-
tadenomas and on the likelihood of these lesions [3].
CT and Magnetic Resonance Imaging(MRI) are excellent
tests for cystic lesions of the pancreas, not only for the ini-
tial detection of a lesion but also for the characterization
of such lesions by visualization of the calcification of the
cyst wall, septa, mural nodules, possible showing of com-
munication between a cyst and the pancreatic duct, and
findings suggestive of pancreatitis. The presence of a cen-
tral scar visualized with the use of CT or MRI is a highly
diagnostic feature that is found in about 20% of serous
cystadenomas [3].
Management of these lesions has not been standardised,
is evolving and depends on many factors such as the pres-
ence or absence of symptoms, surgical risk of the patient,
age, type of the tumour, its location and size. The proper
evaluation and subsequent management of this disease in
patients without symptoms have not been fully defined
[1,3]. The role of surgery is central because it could be cur-
ative when complete resection is possible [4]. PET arising
in association with a serous cystadenoma may reinforce
the recommendation for surgical therapy of cystic
tumours of the pancreas as mixed tumours may be present
[5]. The current death rate among patients undergoing
pancreatic resection in specialized centres is less than 2%
[3].
Conclusion
Combined endocrine and exocrine tumours of the pan-
creas are very rare. The potential of pancreatic ductal stem
cells for biphasic differentiation along the embryologic
developmental line of exocrine and endocrine pancreas
could happen. Cystic lesions of the pancreas are asympto-
matic and mainly discovered incidentally. Imaging stud-
ies such as CT and MRI are excellent tests which help
diagnose these lesions. The role of surgery is central in
managing these tumours, especially if both endocrine and
exocrine tumours coexist, and could be curative if com-
plete resection is performed.
Competing interests
The author(s) declare that they have no competing inter-
ests.
Authors' contributions
AA Performed literature review, drafted and revised man-
uscript. VU Evaluated radiological features and contrib-
uted to radiological part of manuscript, AE Carried out
initial assessment of the patient and helped in draft of
manuscript. HM Contributed to the concept of the report
AB Operated on the patient and corrected the manuscript
for it scientific content. ND Evaluated histopathological
features and contributed histological part.
All authors read and approved the final manuscript.
Acknowledgements
A written consent was obtained from the patient for the publication of this 
report.Page 3 of 4
(page number not for citation purposes)
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Combined endocrine and exocrine tumours of the pancreas

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