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Segmental duodenectomy for gastrointestinal stromal
tumor of the duodenum
Nicolas Christian Buchs, Pascal Bucher, Pascal Gervaz, Sandrine Ostermann, François Pugin, Philippe Morel
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Nicolas Christian Buchs, Pascal Bucher, Pascal Gervaz,
Sandrine Ostermann, François Pugin, Philippe Morel, De-
partment of Surgery, University Hospital Geneva, 1211, Geneva,
Switzerland
Author contributions: Buchs NC, Bucher P and Gervaz P con-
ceived and designed this study; Ostermann S and Pugin F col-
lected the data; Buchs NC, Bucher P and Morel P analyzed and
interpreted the data; Buchs NC, Bucher P and Pugin F wrote
the manuscript; Gervaz P, Ostermann S and Morel P revised the
manuscript critically for important intellectual content; all au-
thors approved the nal version of the article.
Correspondence to: Nicolas Christian Buchs, MD, Depart-
ment of Surgery, University Hospital Geneva, 24 rue Micheli-
du-Crest, 1211, Geneva,
Switzerland. nicolas.c.buchs@hcuge.ch
Telephone: +41-22-3723311 Fax: +41-22-3727707
Received: January 7, 2010 Revised: March 4, 2010
Accepted: March 11, 2010
Published online: June 14, 2010
Abstract
AIM: To evaluate the results of segmental duodenec-
tomy (SD) and pancreaticoduodenectomy (PD) for
duodenal gastrointestinal stromal tumor (GIST) and
help clinicians with surgical management.
METHODS: All patients who underwent surgery for
non-metastatic GIST of the duodenum in a single insti-
tution since 2000 were prospectively followed up. Sev-
en patients (median age 51 years, range: 41-73 years)
were enrolled: ve underwent SD and two underwent
PD.
RESULTS: All the patients had a complete resection
(R0), with no postoperative morbidity and mortality.
Among the SD group, GIST was classied as low risk
in two patients, intermediate risk in two, and high risk
in one, according to the Fletcher scale, (
vs
two high
risk patients in the PD group). With a median follow-
up of 41 (18-85) mo, disease-free survival (DFS) rates
were 100% after SD and 0% after PD (
P
< 0.05). The
median DFS was 13 mo in the PD group.
CONCLUSION: Whenever associated with clear sur-
gical margins, SD is a reliable and curative option for
most duodenal GISTs, and is compatible with long-
term DFS.
© 2010 Baishideng. All rights reserved.
Key words: Gastrointestinal stromal tumor; Duodenal
neoplasms; Segmental duodenectomy; Pancreatico-
duodenectomy
Peer reviewer: Ahmet Tekin, MD, Department of General
Surgery, IMC Hospital, Istiklal Cad no:198, Mersin 33100,
Turkey
Buchs NC, Bucher P, Gervaz P, Ostermann S, Pugin F, Morel P.
Segmental duodenectomy for gastrointestinal stromal tumor of
the duodenum. World J Gastroenterol 2010; 16(22): 2788-2792
Available from: URL: http://www.wjgnet.com/1007-9327/full/
v16/i22/2788.htm DOI: http://dx.doi.org/10.3748/wjg.v16.i22.
2788
INTRODUCTION
Gastrointestinal stromal tumors (GISTs) are the most
common mesenchymal tumors of the digestive tract,
with an estimated annual incidence between 10 and
20/106 people[1,2]. Although GISTs are encountered all
along the digestive tract, the most frequent sites of oc-
currence are the stomach (50%) and small bowel (30%).
Duodenal GISTs are less frequent and account for < 5%
of cases, but still represent approximately 30% of pri-
mary duodenal tumors[3].
Surgery is still the only curative approach for GIST[4-6],
but the optimal surgical procedure for duodenal GIST
remains to be established. A number of authors have
reported various procedures including pancreaticoduo-
BRIEF ARTICLE
World J Gastroenterol 2010 June 14; 16(22): 2788-2792
ISSN 1007-9327 (print)
© 2010 Baishideng. All rights reserved.
Online Submissions: http://www.wjgnet.com/1007-9327ofce
wjg@wjgnet.com
doi:10.3748/wjg.v16.i22.2788
Buchs NC
et al
. Segmental duodenectomy for duodenal GIST
denectomy (PD), pancreas-sparing duodenectomy, seg-
mental duodenectomy (SD), or wedge local resection, but
few have correlated the different options with oncological
results[7-16].
Two major tumor characteristics have to be consid-
ered for surgical resection of duodenal GIST, which
differs from duodenal adenocarcinoma[17]. First, GIST
spreads specically hematogenously and is rarely, if ever,
associated with lymphatic invasion, as in other sarco-
mas[18]. Secondly, GISTs are well encapsulated tumors
that rarely have a tendency to local invasion[4]. For these
reasons, radical lymphadenectomy or extended resection
of adjacent organs should not confer a survival advan-
tage in non-metastatic duodenal GIST[6,19,20].
Therefore, this study was undertaken to audit the
oncological results of segmental duodenal resection in
comparison with more extensive procedure such as PD
for primary non-metastatic duodenal GIST.
MATERIALS AND METHODS
This was a prospective cohort study of all surgical patients
treated in our department for primary non-metastatic
duodenal GIST from 2000 to 2008. Inclusion criteria were
patients presenting with suspicion of non-metastatic duo-
denal GIST in a single hospital (Figures 1 and 2). Exclu-
sion criteria were a clearly metastatic disease, poor health
condition that precluded laparotomy (severe pulmonary
disease, non-treatable coagulation abnormality), and pa-
tient’s refusal to participate in this study. All patients had
complete surgical resection (R0). Seven cases were includ-
ed. Median age was 51 years (range: 41-73 years).
Five patients had an SD and two a cephalic PD. PD
was the operation chosen for relatively large and difcult-
to-reach tumors (rst and second part of the duodenum).
Among the SD group, one patient had a duodenal patch
resection of a small D1 GIST with direct closure (case 3,
Table 1), the others had a complete SD with latero-lateral
duodeno-jejunal reconstruction (Figure 3).
Pathological diagnosis of GIST was confirmed for
all according to histological and immunohistochemical
work-up. All tumors were c-kit positive. GIST was clas-
sied according to the Fletcher scale[21] and our scale[4].
The primary endpoint for this analysis was disease-
free sur vival (DFS), which was defined as time from
surgery to GIST recurrence. Follow-up was available for
all patients at the date set for collecting data, November
2008. Follow-up was carried out through routine visits at
our Outpatient Oncological Clinic. Clinical assessment
was made every 3 mo during the rst 2 years after sur-
gery and every 6 mo thereafter, with detection of recur-
rence as soon as possible to allow adjuvant therapy with
imatinib. Yearly chest X-rays and abdominal computed
tomography (CT) were routinely performed in all pa-
tients and additional imaging was requested when clini-
cal suspicion of GIST recurrence occurred. The median
follow-up was 41 (18-85) mo.
Statistical analysis
Statistical analysis was performed using GraphPad InStat
(GraphPad Software, San Diego, CA, USA). When ap-
propriate, data were analyzed using two-sided Fischer’s
test or two-sided t test. P < 0.05 was considered statisti-
cally signicant.
RESULTS
Study population and pathological data
Seven patients were included in the analysis. Four pa-
tients presented with upper digestive tract hemorrhage,
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Figure 3 Duodeno-jejunal
anastomosis. Latero-lateral
duodeno-jejunal anastomosis
between the second part of the
duodenum and the first jejunal
loop was easily performed after
distal duodenectomy and Ko-
cher maneuver.
Figure 1 Gastrointestinal stromal tumor (GIST) located in the third part of
the duodenum with typical computed tomography (CT) appearance. Note
the typical CT appearance of GIST with an area of necrosis (central cavitations
with surrounding highly vascular tissue) (white arrow: pancreas; black arrow:
GIST).
Figure 2 GIST located ion the horizontal duodenum (patient 5, Table 1).
Note the close relation between the GIST (short arrow) and the pancreas (long
arrow), which was easily dissected during surgery.
the others with abdominal discomfort. In all patients, the
diagnosis of duodenal GIST was made through CT after
it was suspected by endoscopy in those with digestive
bleeding. No preoperative biopsies were performed.
All GISTs were localized without metastases or
peritoneal dissemination. According to the Fletcher clas-
sication, the GISTs were considered as high risk in the
two patients in the PD group, and in the SD group as
high risk in one, intermediate risk in two, and low risk in
two patients (P > 0.05). According to our classication,
the two GISTs in the PD were classied as malignant, as
was one of the ve GISTs in the SD group (P < 0.05).
Postoperative outcomes
No postoperative morbidity and mortality were record-
ed. All patients had complete surgical (R0) resection of
their duodenal GIST. Five patients underwent SD and
two PD (Table 1). GIST-free surgical margins along the
duodenum ranged from 0.5 cm to 3 cm.
Follow-up results
The median follow-up was 41 mo (range: 18-85 mo). Me-
dian follow-up was 58 mo (range: 56-60 mo) and 37 mo
(range: 18-85 mo) for the PD and SD group, respectively.
Two patients in the PD group demonstrated recurrence
with a median disease-free interval of 13 mo, whereas no
recurrence was observed in the SD group (P < 0.05). All
patients are alive and disease free in the SD group with a
median DFS of 37 mo. The two patients with recurrence
after PD presented with liver metastases, which were
treated with imatinib mesylate, and one was also treated
with partial hepatectomy. A statistically significant dif-
ference was detected between the PD and SD group for
DFS (P = 0.048), however, this should be balanced by a
higher rate of malignant GIST and longer median follow-
up in the PD group.
DISCUSSION
The optimal surgical procedure for GISTs of the duo-
denum remains poorly dened in terms of oncological
results. This study was undertaken to compare oncologi-
cal results of SD and the more radical PD. According
to our data, duodenal GIST prognosis is dependent on
tumor malignant potential when clear surgical margins
can be achieved and not on size of surgical margins or
lymphatic dissection. The data presented herein demon-
strate that SD is associated with prolonged DFS.
Surgical resection is still the only curative therapy for
GIST[1,6,19]. GISTs are known to be resistant to chemo-
therapy and radiotherapy, and the recently developed
molecular targeted therapies (imatinib mesylate and suni-
tinib), while being highly effective in disease control, are
not curative[1,5,6]. The optimal surgical procedure for duo-
denal GIST remains poorly dened in terms of oncolog-
ic results. The reports in the literature addressing surgical
procedures for duodenal GIST demonstrate the feasibil-
ity of various surgical procedures: PD, pancreas-sparing
duodenectomy, SD, or wedge local resection[8,9,11-17,22-25].
These papers can help us a little to determine which sur-
gical procedure is optimal in terms of short- and long-
term oncological results. The largest series of duodenal
GISTs (n = 156) evaluated prognosis according to tumor
grade[3]. In this pathological review, around 60% of pa-
tients underwent pancreas-preserving duodenectomy
and 11% had PD, but due to the retrospective nature of
this analysis, no correlation between type of operation
and oncological results were reported. Very recently,
Tien et al[26] have reported their experience, in which they
compared nine PD with 16 limited operations (11 wedge
resections, and ve SDs) for duodenal GIST. They have
shown that the type of operation is not correlated to op-
erative risk or disease recurrence. They have concluded
that limited procedures, like SD, should be attempted for
duodenal GIST without involvement of the papilla of
Vater. Others have reported similar results[10].
The choice of surgical procedure for duodenal GIST
can be guided by the size and exact location of the tu-
mor[6,11,17,25]. However, some principles of GIST surgical
treatment have to be considered by a visceral surgeon
when approaching duodenal GIST[1,4,6,10,17]. First, GISTs
are mesenchymal tumors that behave as other sarcomas
and not like adenocarcinomas[4,18]. GIST spreads speci-
cally hematogenously and is rarely, if ever, associated with
lymphatic invasion, as in other sarcomas[4,6,18]. Therefore,
lymphadenectomy is not recommended[4,11,19]. This seems
true for duodenal GIST as local lymph node invasion has
never been described, even after PD[3,11-13,17]. Secondly,
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Table 1 Patients characteristics and follow-up
Cases Localization Size (cm) Mitosis/50HPF Fletcher grade Surgery Follow-up DFS Second-line therapy
1 D2 6.5 34 HR PD 60 13 Imatinib and
hepatectomy
2 D1 7 19 HR PD 56 12 Imatinib
3 D1 2.5 3 LR SD 85 85 -
4 D2 2 2 LR Atypical
duodenectomy
40 40 -
5 D2-3 10 10 HR SD 37 37 -
6 D3 7 2 IR SD 29 29 -
7 D4 5.5 5 IR SD 18 18 -
D1: First part of the duodenum; D2: Second part of the duodenum; D3: Third part of the duodenum; D4: Forth part of the duodenum; HR: High
risk; IR: Intermediate risk; LR: Low risk GIST according to Fletcher scale; HPF: High-power eld; DFS: Disease free survival.
Buchs NC
et al
. Segmental duodenectomy for duodenal GIST
GISTs are well encapsulated tumors that rarely show a
tendency to local invasion even for high risk tumors[4,6,20].
They should be approached with the intention of per-
forming complete en bloc removal (R0 resection) of the
tumor and surrounding digestive tract tissue[1,4,6,7,10,19]. The
size of surgical margins along the segment of digestive
tract involved are not formally dened, however there is
little submucosal spread in GIST and clear margins of
1 or 2 cm are recommended[4,6,19,20]. When extracapsular
GIST mobilization is possible, there is no need for exten-
sive surgical margins on adjacent organs and peri-tumoral
resection with an intact capsule is sufcient[4,6,19,20].
Segmental or atypical duodenectomy for duodenal
GIST is in accordance with these principles and could be
benecial for patients because it does not involve the ex-
cessive resection and morbidity associated with PD. Af-
ter complete surgical resection, duodenal GIST progno-
sis seems not to be inuenced by the pancreatic margins,
according to the present study and the sparse literature
on the subject. Prognosis is mainly dependent on malig-
nant status, which is determined by size and mitotic rate
(Fletcher scale). This has been clearly shown for duode-
nal GIST in the study by Miettinen et al[3] and was true in
our small series. However, some authors have advocated
the need for PD as pancreatic invasion cannot be ruled
out on preoperative studies[17,27]. Although close contact
between the GIST capsule and pancreas is usually the
rule for large duodenal GISTs on CT (Figures 1 and 2),
this is rarely correlated with pancreatic tumoral invasion,
which allows treatment with pancreas-sparing duodenec-
tomy[12,28,29]. As a result of these considerations, we think
that segmental or atypical duodenectomy is the optimal
procedure for duodenal GIST, as previously proposed by
others[10-13,15,22-25,30]. The exact type of duodenectomy to
perform might be inuenced by GIST size and location,
ranging from wedge resection with primary closure for
small proximal duodenal GIST to SD with duodeno-je-
junal anastomosis for large distal duodenal GIST[11,15,30-32].
One exception to this might be periampullary or ampul-
lary GIST, which can present with jaundice, for which
pancreas-preserving duodenectomy can be challenging
compared to cephalic PD, when the ampulla needs to be
resected to obtain clear surgical margins[8,27].
The present study has several limitations. First, it
could be argued that a higher rate of malignant GIST is
present in the PD group, because in part, PD was chosen
for larger and more difficult-to-reach lesions. Further-
more, a longer median follow-up was available for the
PD group. These two points could counter-balance the
results. Finally, the sample was small, but duodenal GIST
remains a rare tumor. Previous studies published in the
literature have not reported large numbers of patients,
and most of the time, only case reports[8-17,19,22-27,30].
In conclusion, pancreas-preserving segmental or atypi-
cal duodenectomy seems to be a reliable and curative
option in duodenal GIST. Despite being limited in their
extent, these methods of resection, when performed with
negative margins, are compatible with long-term DFS,
and should be preferred, whenever possible, to PD. This
is related to the tumoral characteristics of GIST, which is
generally well encapsulated, even when highly malignant
and with extremely rare lymphatic spread. When clear sur-
gical margins are achieved, prognosis depends on tumoral
malignant potential and not on the extent of the surgical
margins, especially the pancreatic margin, for duodenal
GIST. However, PD remains a good alternative for tu-
mors in the vicinity of the ampulla of Vater.
COMMENTS
Background
Duodenal gastrointestinal stromal tumors (GISTs) are rare primary duodenal
tumors, and there are few guidelines to help the clinician in their surgical
management. Surgery is still the only curative approach for GIST, but the
optimal procedure remains to be established. Although, numerous authors have
reported various surgical procedures, few have correlated their results with
oncological outcomes.
Research frontiers
This study was designed to assess the optimal surgical procedure for
duodenal GIST, and to compare segmental resection with more extensive
pancreaticoduodenectomy (PD).
Innovations and breakthroughs
The authors reported good oncological outcomes with long-term disease-free
survival (DFS) in the segmental duodenectomy (SD) group. Thus, whenever
associated with clear surgical margins, SD is a reliable and curative option for
most duodenal GISTs. However, PD remains a good alternative for tumors in
the vicinity of the ampulla of Vater.
Applications
Segmental resection should be preferred, when possible, to more extensive
procedures for duodenal GIST. However, for tumors located in the vicinity of the
ampulla of Vater, PD remains a good option.
Peer review
The authors evaluated the results of SD (ve cases) and PD (two cases) for
duodenal GIST. The very low number of patients is certainly a weakness of this
study; on the other hand, duodenal GIST is very rare. The authors obtained
good DFS following limited resection (SD) with clear margins.
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S- Editor Wang JL L- Editor Kerr C E- Editor Zheng XM
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Buchs NC
et al
. Segmental duodenectomy for duodenal GIST