INTRODUCTION: Duodenal gastrointestinal stromal tumors are uncommon and a relatively small subset of gastrointestinal stromal tumors whose optimal surgical procedure has not been well defined. Because submucosal spread and local lymph node involvement are infrequent in gastrointestinal stromal tumors, wide margins with routine lymph node dissection may not be required. Various surgical procedures for duodenal gastrointestinal stromal tumor, pancreatoduodenectomy, pancreas-sparing duodenectomy, segmental duodenectomy, or local resection, have been described depending on the size and exact site of the lesion. CASE PRESENTATION: We present the case of a 65-year-old African woman with a giant gastrointestinal stromal tumor involving the second and third portion of the duodenum successfully treated by partial duodenectomy with duodenojejunostomy. This surgical technique is ideal when a gastrointestinal stromal tumor does not involve the ampulla because it avoids a pancreatoduodenectomy, and has not been previously described for the management of this malignancy. Duodenal gastrointestinal stromal tumor should be suspected in any patient with a duodenal wall mass. CONCLUSIONS: Gastrointestinal stromal tumors of the duodenum should be suspected in any patient with a duodenal wall mass. Extramural growth and central ulceration with or without bleeding should alert the endoscopist to the possibility of a duodenal gastrointestinal stromal tumor diagnosis

Abdelmalek Oussaden

Afaf Amarti

Khalid Ait Taleb

Khalid Maazaz

Leila Chbani

Ouadii Mouaqit

Journal of Medical Case Reports

PubMed

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JOURNAL OF MEDICAL
CASE REPORTS
Mouaqit et al. Journal of Medical Case Reports 2013, 7:184
http://www.jmedicalcasereports.com/content/7/1/184CASE REPORT Open AccessA large gastrointestinal stromal tumor of the
duodenum treated by partial duodenectomy with
Roux-en-Y duodenojejunostomy: a case report
Ouadii Mouaqit1*, Leila Chbani2, Khalid Maazaz1, Afaf Amarti2, Khalid Ait Taleb1 and Abdelmalek Oussaden1Abstract
Introduction: Duodenal gastrointestinal stromal tumors are uncommon and a relatively small subset of
gastrointestinal stromal tumors whose optimal surgical procedure has not been well defined. Because submucosal
spread and local lymph node involvement are infrequent in gastrointestinal stromal tumors, wide margins with
routine lymph node dissection may not be required. Various surgical procedures for duodenal gastrointestinal
stromal tumor, pancreatoduodenectomy, pancreas-sparing duodenectomy, segmental duodenectomy, or local
resection, have been described depending on the size and exact site of the lesion.
Case presentation: We present the case of a 65-year-old African woman with a giant gastrointestinal stromal
tumor involving the second and third portion of the duodenum successfully treated by partial duodenectomy with
duodenojejunostomy. This surgical technique is ideal when a gastrointestinal stromal tumor does not involve the
ampulla because it avoids a pancreatoduodenectomy, and has not been previously described for the management
of this malignancy. Duodenal gastrointestinal stromal tumor should be suspected in any patient with a duodenal
wall mass.
Conclusions: Gastrointestinal stromal tumors of the duodenum should be suspected in any patient with a
duodenal wall mass. Extramural growth and central ulceration with or without bleeding should alert the
endoscopist to the possibility of a duodenal gastrointestinal stromal tumor diagnosis.
Keywords: Duodenojejunostomy, Duodenum, Gastrointestinal stromal tumors, SurgeryIntroduction
Only 3% to 5% of gastrointestinal stromal tumors
(GISTs) are located in the duodenum. They are associ-
ated with an increased risk of fatal gastrointestinal
bleeding, which is a primary manifestation [1,2]. A small
intestinal GIST can occur anywhere along the length of
the bowel and can be multiple. The duodenum is in-
volved in about 10% to 20% of small intestinal GISTs [3].
Although duodenal GISTs are similar pathologically to
those involving other organs, they do have some peculiar
features. GISTs in the duodenum pose particular chal-
lenges for diagnosis and management. We describe the
case of a large duodenal GIST including its presentation,* Correspondence: mouaqit.ouadii@gmail.com
1Surgery Department, University Hospital Hassan II, Fez, Morocco
Full list of author information is available at the end of the article
© 2013 Mouaqit et al.; licensee BioMed Centra
Commons Attribution License (http://creativec
reproduction in any medium, provided the ordiagnosis, and the type of surgery performed, as well as
a review of issues related to GISTs in the duodenum.
Case presentation
A 65-year-old African woman presenting with abdom-
inal pain was referred to our hospital. Her medical his-
tory and family history were unremarkable. She had no
history of previous abdominal surgery. On examination
she looked healthy with no clinical jaundice or pallor.
An abdominal examination revealed a large upper ab-
dominal mass with thinned overlying skin. It had min-
imal mobility and was not tender. The rest of the
examination was normal. Her hemoglobin, on admis-
sion, was 7.0g/dL. She was transfused and underwent an
esophagogastroduodenoscopy, which revealed a sub-
mucosal tumor at D2 and D3. A biopsy was obtained
but was reported as nonspecific. A computed tomog-
raphy (CT) scan of the abdomen revealed a 12×13cml Ltd. This is an Open Access article distributed under the terms of the Creative
ommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and
iginal work is properly cited.
Figure 2 Histological findings of the tumor. Histopathology
showing spindle-shaped tumor arranged in palisading pattern.
Mouaqit et al. Journal of Medical Case Reports 2013, 7:184 Page 2 of 4
http://www.jmedicalcasereports.com/content/7/1/184retroperitoneal mass in the region of the head of the
pancreas (Figure 1). There was no evidence of metastases
to her liver or lung. From these radiographic findings, we
diagnosed a submucosal tumor of the duodenum. The pa-
tient underwent an elective exploratory laparotomy. No
evidence of local invasion of the pancreas or of distant
metastases was found. The tumor had greatly decreased in
size, and it was thought that the liquid of the tumor had
probably emptied into the duodenum through a fistula be-
tween the tumor and the duodenum. Considering that the
pancreas and major papilla were not involved, a local re-
section was performed, with a 1cm disease-free margin. A
retrocolic Roux-en-Y loop was then created and the edges
of the defect in the duodenum joined to the jejunal
limb of the Roux-loop by a hand-stitched side-to-side
duodenojejunostomy anastomosis using a 3-0 Vicryl.
In addition, a resection of the right hemicolon was
performed due to tumor infiltration of the right curvature
of the colon. An ileotransversostomy was performed to re-
construct the gastrointestinal passage. The operative time
was 200 minutes and estimated blood loss was 100mL.
Microscopic examination with hematoxylin and eosin
staining of the tumor showed spindle shaped and epitheli-
oid cells with mild nuclear pleomorphism (Figure 2). Im-
munohistochemistry revealed that the cells strongly
expressed CD117 (Figure 3), with focal expression of
CD34 (Figure 4). Therefore, the final histology was con-
sistent with the diagnosis of a duodenal GIST. Based on
the above findings, the tumor was finally diagnosed as a
GIST with high-grade malignancy originating from the
duodenum. A molecular genetic analysis for KIT protein
mutation was not performed because of its unavailability
at our institute. After the operation, the postoperative di-
gestive opacification showed no digestive fistula (Figure 5).Figure 1 Abdominal computed tomography showing the large
retropancreatic tumor.The patient was treated with imatinib. She was doing very
well with no evidence of disease recurrence when she was
last seen 24 months after her operation.
Discussion
GISTs are defined as mesenchymal tumors arising
from the gastrointestinal wall, mesentery, omentum or
retroperitoneum. GISTs can be located anywhere in
the gastrointestinal tract. The most common sites are
stomach (40% to 60%) and small intestine (30% to
40%) [4]. The mean age of patients with GIST is 53
years. Only about 5% of GIST patients are younger
than 30 years [4]. GISTs of the duodenum make up
only 4.5% of all GISTs [5] and therefore represent a
rare tumor entity. Duodenal GISTs are mainly located
in the second portion of the duodenum (42 out of 156)
and about half of them are malignant [4]. The woman de-
scribed in this case report presented with a duodenal GIST
as another rare GIST manifestation. On presentation, 41%Figure 3 Immunohistochemistry showing positivity for CD117.
Figure 4 Immunohistochemistry. The tumor cells react positively
for CD34.
Mouaqit et al. Journal of Medical Case Reports 2013, 7:184 Page 3 of 4
http://www.jmedicalcasereports.com/content/7/1/184to 47% of malignant GISTs are metastatic [6]. These tu-
mors grow expansively without being invasive and some-
times metastasize to the liver or recur locally. A duodenal
GIST is usually asymptomatic when small in size and can
reach a large size before causing any symptoms. As the
tumor enlarges it causes variable symptomatology. The
most common presentation is gastrointestinal bleeding
which may be chronic and mild or sudden and massive [6].
Although our patient had a large tumor, she had mildFigure 5 The postoperative digestive opacification.anemia. The next most common presentations are abdom-
inal discomfort, pain and swelling [7]. Compared to other
tumor localizations duodenal GISTs manifest with tumor-
associated bleeding in 75% compared to 54% stomach and
28% ileojejunal. In contrast to other localizations duodenal
GISTs are thus associated with a dramatically increased
risk of upper intestinal bleeding [8]. Diagnosis can be made
with upper gastrointestinal endoscopy [6]. The tumor is
usually exophytic, and appears as a submucosal swelling.
Sometimes it presents only as an ulcer, as in our case. The
biopsy should be deep, but may not always be diagnostic.
Endoscopic ultrasound can help in delineating the sub-
mucosal tumor. Alternative diagnostic means include CT,
magnetic resonance imaging (MRI), barium study or ultra-
sonography [9]. However, CT and MRI seem to be the best
imaging modalities for assessment of the primary lesion
and detection of metastases [10]. However, CT scans are
not always helpful in specifying the origin of the mass. In
several cases reported in the literature, the mass was
misdiagnosed as arising from the head of the pancreas
[11]. There is currently uniform agreement that the surgi-
cal treatment of choice for GISTs is resection of the tumor
with clear surgical margins including adjacent organs as
necessary [10]. As local and regional lymph node involve-
ment is infrequent in GIST, routine lymph node dissection
is not advocated [2,3,7,9] and limited resection is fre-
quently performed. The surgical choice depends not only
on the size of the tumor but also on the location in the
duodenal wall and the relation to the ampulla of Vater
[10,12,13]. Patients with duodenal GISTs close to papilla of
Vater should be treated by pancreatoduodenectomy. Vari-
ous techniques of limited resection for duodenal GISTs
have been advocated depending on the site and the size of
the tumors. Wedge resection with primary closure can be
performed for small lesions if the resulting lumen is ad-
equate and the ampulla can be preserved [14,15]. Seg-
mental duodenectomy with side-to-end or end-to-end
duodenojejunostomy can be performed for larger tumors lo-
cated at the third and fourth portion of the duodenum [14].
Partial duodenectomy with Roux-en-Y duodenojejunostomy
can be performed for larger tumors involving the
antimesenteric border of the second and third portion of the
duodenum [16]. Although a limited operation procedure,
such as wedge or segmental resection, is relatively simple to
perform, there is a risk of subsequent anastomotic leakage
or stenosis development, as well as later tumor recurrence
in patients treated by limited operation. By contrast,
pancreatoduodenectomy as a treatment for duodenal GISTs
can provide a wider surgical margin but may be associated
with excessive morbidity, especially in patients with a tumor
of low-grade malignancy [17]. It is not clear what the opti-
mal surgical margin should be, but a negative one is essen-
tial to prevent local recurrence of the tumor. No lymph
node dissection is required because they are very unlikely to
Mouaqit et al. Journal of Medical Case Reports 2013, 7:184 Page 4 of 4
http://www.jmedicalcasereports.com/content/7/1/184be involved [14,18]. The outcome depends on the patho-
logical features of the tumor and the completeness of surgi-
cal resection. Large tumors with high mitotic counts behave
much worse than small tumors with low mitotic counts,
which are considered benign [11]. Local recurrence is higher
in tumors not completely removed or with a positive micro-
scopic margin. After a review of recent literature the duo-
denal tumor localization in our case is thus associated with
a better prognosis, but with an increased bleeding probabil-
ity [8]. These results are in line with the authors' opinion
that primary surgery could be the safest therapeutic option
for a GIST of this localization. Beside the increased risk of
tumor bleeding, caused by the localization, neoadjuvant
imatinib therapy would additionally lead to a higher percent-
age of patients with tumor bleeding. For our patient, there
was no indication of adjuvant treatment because the patient
presented with severe anemia due to tumor bleeding.Conclusions
Duodenal GIST should be suspected in any patient with
a duodenal wall mass. Extramural growth and central ul-
ceration with or without bleeding should alert the en-
doscopist to the possibility of this diagnosis. There is
more than one surgical approach available, but the abso-
lute requirement is complete surgical excision. Preopera-
tive imatinib mesylate can be considered in unresectable
or borderline resectable cases.Consent
Written informed consent was obtained from the patient
for publication of this manuscript and accompanying
images. A copy of the written consent is available for re-
view by the Editor-in-Chief of this journal.
Abbreviations
CT: Computed tomography; GIST: Gastrointestinal stromal tumor;
MRI: Magnetic resonance imaging.
Competing interests
The authors declare that they have no competing interests.
Authors’ contributions
All of the authors were involved in the preparation of this manuscript. OM
performed the operation and revised the manuscript. LC described
histological finding and was involved in drafting the manuscript. All authors
read and approved the final manuscript.
Acknowledgements
We acknowledge our patient for providing informed consent for this case
report.
Author details
1Surgery Department, University Hospital Hassan II, Fez, Morocco.
2Department of Pathology, University Hospital Hassan II, Fez, Morocco.
Received: 10 February 2013 Accepted: 31 May 2013
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doi:10.1186/1752-1947-7-184
Cite this article as: Mouaqit et al.: A large gastrointestinal stromal tumor
of the duodenum treated by partial duodenectomy with Roux-en-Y
duodenojejunostomy: a case report. Journal of Medical Case Reports
2013 7:184.


A large gastrointestinal stromal tumor of the duodenum treated by partial duodenectomy with Roux-en-Y duodenojejunostomy: a case report

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A large gastrointestinal stromal tumor of the duodenum treated by partial duodenectomy with Roux-en-Y duodenojejunostomy: a case report

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