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Segmental duodenectomy for gastrointestinal stromal tumor of the duodenum

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To evaluate the results of segmental duodenectomy (SD) and pancreaticoduodenectomy (PD) for duodenal gastrointestinal stromal tumor (GIST) and help clinicians with surgical management. All patients who underwent surgery for non-metastatic GIST of the duodenum in a single institution since 2000 were prospectively followed up. Seven patients (median age 51 years, range: 41-73 years) were enrolled: five underwent SD and two underwent PD. All the patients had a complete resection (R0), with no postoperative morbidity and mortality. Among the SD group, GIST was classified 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. Whenever associated with clear surgical margins, SD is a reliable and curative option for most duodenal GISTs, and is compatible with long-term DFS.
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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 classied 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/1007-9327ofce
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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 specically 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 difcult-
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-
sied 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 signicant.
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-
sication, 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 classication,
the two GISTs in the PD were classied 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 dened 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 dened 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 dened, 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 sufcient[4,6,19,20].
Segmental or atypical duodenectomy for duodenal
GIST is in accordance with these principles and could be
benecial 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 inuenced 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 inuenced 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
... Unlike adenocancers, GISTs do not tend to lymphatic metastases [5], and local invasion into surrounding tissues is rare [4]. For the reasons listed, radical lymph dissection or wide resections do not contribute to disease-free survival in nonmetastatic cases [6]. In this case report, a case of nonmetastatic GIST located on the anterior wall of the 2nd part of the duodenum was evaluated. ...
... Duodenal GISTs are seen at a rate of approximately 3-5% [7], and are most commonly located in the 2nd part of the duodenum [3]. GISTs have a malignant potential of 10-30%, and a chance of cure can still be achieved with surgical resection [6]. Like other sarcomas, GISTs are resistant to chemotherapy and radiotherapy, and currently available molecular targeted drugs such as imatinib and sunitinib provide disease control but are not curative. ...
... Like other sarcomas, GISTs are resistant to chemotherapy and radiotherapy, and currently available molecular targeted drugs such as imatinib and sunitinib provide disease control but are not curative. In cases of duodenal GIST, surgical treatment alternatives such as PD, pancreatic-sparing duodenectomy (PSD), segmental duodenectomy (SD), wedge local resections are available and can be applied with conservative or laparoscopic/robotic minimally invasive methods [6,7]. GISTs are mesenchymal tumors and, like other sarcomas, lymphatic metastases are rare, so they do not require lymphatic dissection [4,5]. ...
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The most common location of gastrointestinal stromal tumors (GISTs) in the digestive system is the stomach with a rate of 50-70% and they are only seen in the duodenum at a rate of 3-5%. Due to the complex anatomical structure and localization of the duodenum, it is a region where complications are more common in surgical treatment compared to other localizations of the gastrointestinal tract. In this study, a 56-year-old male patient who underwent wedge resection in a case of GIST located in the 2nd part of the duodenum is presented.
... However, there is a paucity of studies comparing the oncological outcome and overall survival of both the procedures. [3] We report a case of GIST of the distal duodenum in a patient who presented predominantly with anaemia, and treated by pancreas-sparing distal duodenectomy. ...
... Beham et al., [13] in their single centre experience with 13 patients, concluded that the type of operative procedure did not affect the long-term survival and the choice of procedure should depend on resectability and patients' performance status. Similar results were published by Buchs, [3] showing segmental duodenectomy to be a curative option with comparable disease-free survival for most duodenal GISTs. ...
Article
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Although gastrointestinal stromal tumours (GISTs) are encountered all along the gastrointestinal tract, duodenal GISTs are uncommon and account for <5% of the cases. A 45-year-old woman presented chiefly with anaemia and associated symptoms, whom on further evaluation was found to have a non-metastatic GIST in the distal duodenum sparing the pancreas and major vasculature. Patient was undertaken for segmental duodenectomy with the help of advanced bipolar energy device (tumour occupying D3–D4 with 1 cm proximal margin and 15 cm jejunum) preserving the pancreas and ampulla with end-to-end duodenojejunostomy with an uneventful postoperative course and clear margins on histopathology. Thus, the patient underwent a less morbid procedure with satisfactory oncological outcome and early resumption of activity. This highlights the need to conduct more trials to gather high level evidence in favour of conservative resection and its oncological adequacy and impact on overall survival and recurrence.
... Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, accounting for 1-3% of all gastrointestinal malignancies [1]. Duodenal stromal tumors are rare tumors among GISTs, accounting for 4-5% of GISTs [2]. Most of them develop in people over 40 years old, and there is no obvious sex difference [3]. ...
... comprehensively consider the location, volume, and nature of the tumor, and the extent of invasion of surrounding tissues. The surgical principle of duodenal GISTs should be complete tumor resection to avoid tumor rupture and implantation metastasis [2,6]. For duodenal GISTs in special locations requiring combined organ resection, if it is difficult to perform R0 resection, imatinib can be used for preoperative treatment [7]. ...
Article
Introduction: Gastrointestinal stromal tumors (GISTs) rarely occur in the duodenum, and only a few cases have been reported. Its clinical manifestations are not specific, and the imaging examination results are not typical, so a preoperative diagnosis is difficult. Pathologic examinations and genetic testing after surgical resection are the main diagnostic methods. Here, a case of duodenal stromal tumor complicated by gastrointestinal perforation is reported. A 57-year-old man presented with paroxysmal abdominal pain and bloating for 7 days. Contrast-enhanced computed tomography of the abdomen revealed a large mass (10 cm in diameter) in the right upper abdomen, which was considered neoplastic. The mass was anterior and inferior to the head of the pancreas, and medial to the mesenteric vessels. The tumor surrounded the descending and horizontal parts of the duodenum, and it ruptured into the lumen of the descending duodenum. After the patient underwent tumor resection, we found a rupture of the descending duodenal opening. After that, duodenal fistula drainage, gastrostomy, jejunostomy, small intestinal adhesion release and abdominal irrigation drainage were performed. Immunohistochemical staining results were as follows: CD34 (-), desmin (-), S-100 (-), CD117 (9.7) (+), DoG-1 (+), SDHB (+), Ki-67 (+5%). Based on these results, the lesion was finally diagnosed as duodenal GIST. The patient underwent surgical resection without targeted therapy and recovered well. Discussion: Duodenal stromal tumors often present with gastrointestinal bleeding and other clinical symptoms, requiring urgent surgery. Complete resection of the tumor is an effective surgical method. Extended resection does not prolong survival. However, surgical treatment should be determined according to the size and location of the tumor and its relationship to the pancreas. This highly malignant duodenal stromal tumor was >10 cm, accompanied by gastrointestinal perforation and necrosis. Surgical resection was required while protecting the organ function.
... Based on the tumor location and spread, patients with GIST can either be asymptomatic or present with various clinical symptoms, for example, intestinal obstruction and hemorrhage [3]. Taking into consideration the location, volume, and nature of the tumor, as well as the extent of invasion of surrounding tissues, the preferred and effective treatment for GIST is surgical management [4]. Gallstones in concurrence with GIST are an extremely rare condition. ...
Article
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Gastrointestinal stromal tumors (GISTs) are considered the most common mesenchymal tumors of the digestive system. However, they make up less than 1% of all GI tumors. GISTs arise from the interstitial cells of Cajal and are commonly found in the stomach and small intestine, and rarely in the colon and esophagus. In this case report, we present a 57-year-old male, a known diabetic, who complained of abdominal pain. He was diagnosed with cholelithiasis and a GIST in the jejunum, which was managed laparoscopically without complications. Most of the time, patients with GIST present with vague symptoms, or sometimes, they are asymptomatic. The most common symptoms are abdominal pain, GI bleeding, and an abdominal mass. These symptoms are usually related to the site of tumor growth, tumor size, and tumor rupture or perforation. Regardless of the tumor location, surgical excision is the gold standard for treating GISTs.
... GISTs are relatively common mesenchymal tumors that occur predominantly in the stomach (60-70%) and small intestine (25-35%) [3]. dGISTs are rare lesions, c EUS fine-needle aspiration (FNA) considered, but was difficult to perform, because of a pulsating blood vessel present in the region to be punctured constituting 30% of primary duodenal tumors and less than 5% of all GISTs [4]. On CT, dGISTs appear as heterogeneously enhanced hypervascularized masses [5,6]. ...
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Background Duodenal gastrointestinal stromal tumors are rare. If tumor growth is extraluminal and involves the head of the pancreas, the diagnosis of a duodenal gastrointestinal stromal tumor is difficult. Case presentation A 44-year-old Japanese woman was referred to our hospital with anemia. An enhanced computed tomography scan showed a hypervascular mass 30 mm in diameter, but the origin of the tumor, either the duodenum or the head of the pancreas, was unclear. Upper gastrointestinal endoscopy revealed bulging accompanied by erosion and redness in part of the duodenal bulb. Mucosal biopsy was not diagnostic. Endoscopic ultrasound fine-needle aspiration was difficult to perform because a pulsating blood vessel was present in the region to be punctured. These findings led to a diagnosis of pancreatic neuroendocrine tumor invasion to the duodenum. The patient underwent pancreaticoduodenectomy. Histologically, the tumor was made up of spindle-shaped cells immunohistochemically positive for c-Kit and CD34. The tumor was ultimately diagnosed as a duodenal gastrointestinal stromal tumor. Conclusion Extraluminal duodenal gastrointestinal stromal tumors are rare and mimic pancreatic neuroendocrine tumors. Endoscopic ultrasound fine-needle aspiration is useful for preoperative diagnosis, but it is not possible in some cases. Intraoperative diagnosis based on a completely resected specimen of the tumor may be useful for modifying the surgical technique.
... En los pacientes con GIST primarios, la resección quirúrgica ofrece una tasa de supervivencia a los cinco años de 48 a 70%. 27 A diferencia de los GIST localizados en otros lugares del tubo digestivo, el tratamiento quirúrgico óptimo para los duodenales no se encuentra bien caracterizado y su establecimiento es escaso en la literatura quirúrgica. 28 La resección quirúrgica puede llevarse a cabo mediante varias opciones. ...
Article
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Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasms, although annual reported incidence rates worldwide are less than 20 per million. These tumors are a primary gastrointestinal disease that can arise anywhere in the digestive tract in adults. Duodenal lesions represent approximately 3 to 5%, although duodenal gastrointestinal stromal tumors are relatively rare, they account for almost 30% of all primary tumors of the duodenum, they originate most frequently in the second portion of the duodenum, followed in order by the third, fourth and first portion. We present the case of a 54-year-old patient with a history of neurofibromatosis, which occurred with episodes of bleeding of three years of evolution, with diagnosis of duodenal polyp; the histopathological study reported gastrointestinal stromal tumor, with an immunohistochemical study that was positive for CD117, CD34 and DOGL, with an account of less than 2 mitosis / 50 CAP. Surgical management was decided, a 3-cm tumor was identified in the lateral wall of the duodenum. In the second portion, wedge resection and primary closure were performed in two suture lines. Local resection is appropriate when feasible and pancreaticoduodenectomy should be reserved for lesions that are not amenable to local resection.
Article
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La localización en el duodeno es la más compleja para el tratamiento de los tumores del estroma gastrointestinal (Gastrointestinal Stromal Tumors, GIST). Los GIST duodenales son relativamente infrecuentes, con una prevalencia de 5 % a 7 % de todos los tratados quirúrgicamente. La mayoría de las publicaciones sobre GIST duodenales son reportes de caso o series de casos. Consecuentemente, las manifestaciones clínicas, el diagnóstico radiológico, el tratamiento quirúrgico y los factores pronósticos, constituyen materia de controversia. En el presente artículo se revisa, principalmente, el tratamiento quirúrgico de los GIST duodenales, tratando de establecer las opciones quirúrgicas de acuerdo con su localización. La mayoría de los artículos sobre GIST duodenales mencionan que, a diferencia de otros tumores localizados en el aparato gastrointestinal, el procedimiento óptimo para el tratamiento del GIST duodenal no se encuentra bien caracterizado en la literatura científica. Sin embargo, la revisión de las publicaciones sobre el tema demuestra que el abordaje quirúrgico descrito por diferentes autores es bastante estándar. Todos toman en cuenta la localización del GIST en el duodeno y sus relaciones anatómicas, para decidir entre la resección local o la pancreatoduodenectomía. Utilizando este conocimiento de sentido común, se proponen opciones quirúrgicas para GIST duodenales basadas en su localización en el duodeno.
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Controlled microrobotic navigation inside the body possesses significant potential for various biomedical engineering applications. Successful application requires considering imaging, control, and biocompatibility. Interaction with biological environments is also a crucial factor in ensuring safe application, but can also pose counterintuitive hydrodynamic barriers, limiting the use of microrobots. Surface rolling microrobots or surface microrollers is a robust microrobotic platform with significant potential for various applications; however, conventional spherical microrollers have limited locomotion ability over biological surfaces due to microtopography effects resulting from cell microtopography in the size range of 2–5 µm. Here, the impact of the microtopography effect on spherical microrollers of different sizes (5, 10, 25, and 50 µm) is investigated using computational fluid dynamics simulations and experiments. Simulations revealed that the microtopography effect becomes insignificant for increasing microroller sizes, such as 50 µm. Moreover, it is demonstrated that 50 µm microrollers exhibited smooth locomotion ability on in vitro cell layers and inside blood vessels of a chicken embryo model. These findings offer rational design principles for surface microrollers for their potential practical biomedical applications.
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Surface microrollers have emerged as a promising microrobotic platform for navigation in the circulatory system as future drug/gene delivery applications. The circulatory system comprises various vessels with different dimensions, blood flow velocities, and flow regimes. Therefore, the performance of surface microrollers would vary in blood vessels. Herein, the performance of surface microrollers, with diameters between 5 and 50 μm, inside vessels of the systemic circulation including veins, venules, capillaries, arterioles, and arteries is investigated with computational fluid dynamics simulations. The simulation environment consists of a simplified fluid with the viscosity and density of blood, without red blood cells, in a cylindrical pipe. The microrollers demonstrate successful upstream locomotion ability in veins and partially in arteries but fail to perform in smaller blood vessels due to significant confinement and flow effects. Overall, the results presented here establish a preliminary result for the future in vivo use of surface microrollers.
Article
Background The second part of the duodenum is the most common part to be involved with duodenal gastrointestinal tumors (D2-GISTs). Localized resection (LR) and pancreaticoduodenectomy (PD) are two viable options for curative resection. The aim of this study is to compare the middle-term outcomes in patients with D2-GIST after either LR or PD in a single institution. Patients and Methods Overall, 53 patients with non-metastatic D2-GIST were analyzed. Either LR or PD was executed depending on the involvement of the ampulla of Vater. The tumors were stratified in accordance with the Miettinen classification for tumor behavior. The patients were followed up for 3 years for recurrence and survival. Results Thirty-two of the patients were females (60%) and 21 males (40%), with a mean age of 55 ± 8 years. Bleeding was the most common presentation in 19 patients (36%). LR was performed in 41 patients (77%), whereas PD was performed in 12 patients (23%). Three-year survival and recurrence were comparable between the two groups. The disease-free survival at 3 years was 85% and 92% in LR and PD group, respectively. The PD group had a significantly longer operative time and a higher incidence of postoperative pancreatic fistula. Otherwise, no statistically significant difference was calculated. A significantly shorter survival was calculated in those with a mitotic index of >5 and also for tumors classified as high grade in accordance with the Miettinen classification. 71% of those with recurrence had high mitotic index > 5/hpf. Conclusions LR for D2-GIST is an acceptable alternative to PD with satisfactory middle-term outcomes. For tumors involving the ampulla of Vater, PD is still indicated. Furthermore, tumor biology predicts the likelihood of survival and recurrence.
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Gastrointestinal stromal tumours (GIST) are the most common mesenchymal tumours of the digestive tract. Most gastrointestinal soft tissue neoplasms, previously classified as leiomyomas, schwannomas, leiomyoblastomas or leiomyosar- comas, are today classified as GIST on the basis of molecular and immunohistological features. They originate from gastrointestinal pacemaker cells and are characterised by over-expression of the ty- rosine kinase receptor KIT. Overall 5-year survival after surgical resection of GIST is approximately 60%. However, these tumours span a wide clinical spectrum from benign to highly malignant. Prog- nostic factors have recently been identified for GIST and include tumour size, mitotic rate and other minor factors. At present, surgery is the stan- dard treatment for primary resectable GIST. Benign GIST have an excellent prognosis after primary surgical treatment, with over 90% 5-year survival. While recurrent or malignant GIST, which are resistant to radiotherapy and chemo- therapy, had until recently an extremely poor prognosis even after surgical resection, with me- dian survival of 12 months. The development of a tyrosine kinase inhibitor has changed the manage- ment of unresectable malignant cases. This new tyrosine kinase inhibitor, imatinib mesylate, which inhibits the c-kit receptor, has proved highly ef- fective against GIST and has improved survival in metastatic GIST. This paper reviews the literature and our experience of GIST, including: diagnosis, pathology, treatment and prognosis.
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As a result of major recent advances in understanding the biology of gastrointestinal stromal tumors (GISTs), specifically recognition of the central role of activating KIT mutations and associated KIT protein expression in these lesions, and the development of novel and effective therapy for GISTs using the receptor tyrosine kinase inhibitor STI-571, these tumors have become the focus of considerable attention by pathologists, clinicians, and patients. Stromal/mesenchymal tumors of the gastrointestinal tract have long been a source of confusion and controversy with regard to classification, line(s) of differentiation, and prognostication. Characterization of the KIT pathway and its phenotypic implications has helped to resolve some but not all of these issues. Given the now critical role of accurate and reproducible pathologic diagnosis in ensuring appropriate treatment for patients with GIST, the National Institutes of Health convened a GIST workshop in April 2001 with the goal of developing a consensus approach to diagnosis and morphologic prognostication. Key elements of the consensus, as described herein, are the defining role of KIT immunopositivity in diagnosis and a proposed scheme for estimating metastatic risk in these lesions, based on tumor size and mitotic count, recognizing that it is probably unwise to use the definitive term "benign" for any GIST, at least at the present time.
Article
As a result of major recent advances in understanding the biology of gastrointestinal stromal tumors (GIST), specifically recognition of the central role of activating KIT mutations and associated KIT protein expression in these lesions, and the development of novel and effective therapy for GISTs using the receptor tyrosine kinase inhibitor STI-571, these tumors have become the focus of considerable attention among pathologists, clinicians, and patients. Stromal/mesenchymal tumors of the gastrointestinal tract have long been a source of confusion and controversy with regard to classification, line(s) of differentiation, and prognostication. Characterization of the KIT pathway and its phenotypic implications has helped to resolve some but not all of these issues. Given the now critical role of accurate and reproducible pathologic diagnosis in ensuring appropriate treatment for patients with GIST, the National Institutes of Health (NIH) convened a GIST workshop in April 2001 with the goal of developing a consensus approach to diagnosis and morphologic prognostication. Key elements of the consensus, as described herein, are the defining role of KIT immunopositivity in diagnosis and a proposed scheme for estimating metastatic risk in these lesions, based on tumor size and mitotic count, recognizing that it is probably unwise to use the definitive term benign for any GIST, at least at the present time.
Article
IntroductionPresent surgical opinion is divided regarding the optimal method for the treatment of duodenal gastrointestinal stromal tumor (GIST) with some supporting the selective use of limited resection (LR) versus others who prefer pancreaticoduodenectomy (PD).MethodsA retrospective review of 22 patients who underwent surgery for suspected GIST involving the duodenum.ResultsThere were 15 GISTs, 1 leiomyosarcoma and 6 other non-GIST benign submucosal tumors. Seven patients underwent LR and seven underwent PD for GIST. The median follow-up was 42 (range, 2–174) months. Patients who underwent LR versus PD had similar mean disease-specific survival [144 (95% CI, 92–196) vs. 130 (95% CI, 82–127) months, P = 0.808] and recurrence rates (14% vs. 29%, P = 0.515). All recurrences occurred at distant sites. Comparison between LR versus PD demonstrated that LR was associated with a significantly shorter operation time [125 (range, 50–305) vs. 350 (range, 210–465) min., P = 0.001] but similar morbidity rate (23% vs. 43%, P = 0.357). Comparison between GIST and other benign tumors demonstrated that size was the only statistically significant distinguishing factor [8.5 (range, 2.5–18.0) vs. 2.5 (range, 1.5–8.0) cm, P = 0.014].Conclusion Benign non-GIST tumors may be distinguished from duodenal GIST as they are smaller in size. LR is a viable treatment option for suspected GIST involving the duodenum. J. Surg. Oncol. 2008;97:388–391. © 2007 Wiley-Liss, Inc.
Article
Duodenal gastrointestinal stromal tumors (GISTs) represent a unique dilemma with varied surgical treatment options. However, the impact of operative methods on disease recurrence has never been addressed. We retrospectively reviewed the medical records of all patients with duodenal GISTs treated at our hospital from January 2001 to December 2008. Of the 25 patients included for analysis, 9 had pancreaticoduodenectomy (PD) and 16 had limited operation. Comparison of clinicopathological data between tumors treated by PD and by limited operation showed no significant differences in patient age, sex, symptoms, location of tumor, tumor grade, immunohistochemical staining pattern, or complications after surgery. However, patients with tumors >5 cm (P = 0.005) or not diagnosed as GISTs before surgery (P = 0.004) were significantly more frequently treated by PD. In multivariable analysis, the only significant predictor for disease recurrence was high-risk duodenal GISTs. Based on the fact that type of operation was not correlated to operative risk and disease recurrence, limited operation rather than PD should be attempted for duodenal GIST without involvement of papilla of Vater to preserve more pancreas parenchyma, duodenum, and common bile duct.
Article
Side to side duodenojejunostomy is a reasonable alternative to other methods of reconstitution of the continuity of the upper portion of the GI tract in selected circumstances. It is not necessary to use duodenojejunostomy in resections distal to the ligament of Treitz because an end to end anastomosis may be readily accomplished. The described technique, however, provides a convenient means of reconstruction for the upper portion of the GI. tract when resection of the small intestine is necessary at the ligament of Treitz or in close proximity to the origin of the mesenteric vasculature. Thus, adequate resection at the ligament of Treitz may be performed when necessary, and the need to subsequently visualize or mobilize the transected duodenal stump to construct an anastomosis in that region is obviated.
Article
To analyze the outcome of 200 patients with gastrointestinal stromal tumor (GIST) who were treated at a single institution and followed up prospectively. A GIST is a visceral sarcoma that arises from the gastrointestinal tract. Surgical resection is the mainstay of treatment because adjuvant therapy is unproven. Two hundred patients with malignant GIST were admitted and treated at Memorial Hospital during the past 16 years. Patient, tumor, and treatment variables were analyzed to identify patterns of tumor recurrence and factors that predict survival. Of the 200 patients, 46% had primary disease without metastasis, 47% had metastasis, and 7% had isolated local recurrence. In patients with primary disease who underwent complete resection of gross disease (n = 80), the 5-year actuarial survival rate was 54%, and survival was predicted by tumor size but not microscopic margins of resection. Recurrence of disease after resection was predominantly intraabdominal and involved the original tumor site, peritoneum, and liver. GISTs are uncommon sarcomas. Tumor size predicts disease-specific survival in patients with primary disease who undergo complete gross resection. Tumor recurrence tends to be intraabdominal. Investigational protocols are indicated to reduce the rate of recurrence after resection and to improve the outcome for patients with GIST.
Article
Pancreas-sparing duodenectomy (PSD) is a safe and effective operative procedure for patients with nonmalignant duodenal polyps. Retrospective analysis of outcomes in patients undergoing PSD. A tertiary referral center. All patients undergoing PSD at the Mayo Clinic, Rochester, Minn. Indications were the presence of numerous duodenal polyps or large, solitary, adenomatous polyps not amenable to endoscopic resection. Dysplasia without frank malignancy was demonstrated in all patients by endoscopic biopsy specimens. Follow-up was complete in all patients. Operative feasibility, short- and long-term complications, quality of life, and survival. Five patients had diffuse polyposis (familial adenomatous polyposis) and 3 had very large periampullary villous adenomas. The mean age of the patients was 54 years (female-male ratio, 3:1). Colectomy preceded PSD in 5 patients (familial adenomatous polyposis); 3 had previous transduodenal excision of adenomas, and 2 had previous resections of desmoid tumors. The mean operating room time was 370 minutes; blood loss, 340 mL; and the length of the hospital stay, 18 days. All specimens showed dysplasia (5 low grade, 3 high grade). There were 5 major complications--3 ampullary leaks that closed spontaneously with drainage, 1 intra-abdominal hemorrhage requiring a second exploratory surgical procedure, and 1 deep wound infection. The mean follow-up was 23 months. All patients experienced weight gain and good performance status. A second endoscopy (performed in 5 patients) demonstrated small polyps in the neoduodenum in 2 patients and tiny anastomotic ulcers in 2 patients. For 1 patient, there were no abnormalities seen on the endoscopy. Two patients have since developed transient bouts of pancreatitis. Pancreas-sparing duodenectomy, although technically demanding, eliminates the need for pancreatic resection. Pancreas-sparing duodenectomy is associated with good absorptive capacity, weight gain, and quality of life. Furthermore, it may reduce the risk of subsequent malignancy. Long-term surveillance, however, is still required. Pancreas-sparing duodenectomy is contraindicated in the setting of malignancy.
Article
Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. Until recently, surgery has been the only effective therapy for GIST. However, even after complete resection of tumor, many patients still eventually die of disease recurrence. Conventional chemotherapy and radiation therapy have been of limited value. Within the last few years, it was discovered that most GISTs have a gain-of-function mutation in the c-kit proto-oncogene. This results in ligand-independent activation of the KIT receptor tyrosine kinase and an unopposed stimulus for cell growth. STI-571 is a small molecule that selectively inhibits the enzymatic activity of the ABL, platelet-derived growth factor receptor, and KIT tyrosine kinases and the BCR-ABL fusion protein and is a landmark development in cancer therapy. Its clinical development marks a new era of rational and targeted molecular inhibition of cancer that emanates from direct collaborations between scientists and clinicians. It provides proof of the principle that a specific molecular inhibitor can drastically and selectively alter the survival of a neoplastic cell with a particular genetic aberration. The advent of STI-571 has markedly altered the clinical approach to GIST. It has proven to be effective in metastatic GIST and is also under investigation as a neoadjuvant and adjuvant therapy.