A rare giant gastrointestinal stromal tumor of the stomach traversing the upper abdomen: A case report and literature review
This article presents a 66-year-old woman with a huge GIST of stomach that traverses the upper abdomen. With only the predominant abdominal sign featuring a palpable huge mass, but none special physical findings and routine blood as well as biochemical markers, it could hardly judge where the mass drive from upon the images. Furthermore, many important blood vessels had a complex relationship with the mass according to radiological findings. An exploratory laparotomy revealed a huge tumor that protrude from prior wall of stomach fundus, lesser curvature of stomach side, measuring approximately 21 x 34 x 11 cm in diameter and weighing 5.5Kg. A completely resection was decided on the tumor and the department of pathology immunohistochemically characterized it (CD117, CD34, Dog-1, etc) as a GIST of stomach. Preoperatively diagnosis of GISTs is still sometimes puzzling. Hoping the presentation of this rare case and the literature review could benefit others in similar problems.
C ASE R E P O R T Open Access
A rare giant gastrointestinal stromal tumor of the
stomach traversing the upper abdomen: a case
report and literature review
Lei Zhou, Chang Liu
, Ji-Gang Bai, Ji-Chao Wei, Kai Qu, Feng Tian, Ming-Hui Tai, Rui-Tao Wang and Fan-Di Meng
We present the case of a 66-year-old woman with a huge gastrointestinal stromal tumor of the stomach that
traversed her upper abdome n. The predominant abdominal sign was a huge, palpable mass, but there were no
other distinctive findings in her physical examination or her routine blood workup, including biochemical markers. It
was difficult to judge the origin of the mass upon imaging. Furthermore, radiological findings revealed that the
mass had a complex relationship with many major blood vessels. An exploratory laparotomy revealed a hu ge tumor
protruding from the anterior wall of the stomach fundus, on the lesser curvature of the stomach, measuring
approximately 21 × 34 × 11 cm in diameter and weighing 5.5 kg. A complete resection was performed and the
tumor was characterized on immunohistochemistry as a gastrointestinal stromal tumor of the stomach. Preoperative
diagnosis of gastrointestinal stromal tumors can be difficult, and we hope that the presentation of this rare case
and literature review will benefit other diagnosing clinicians having similar problems.
Keywords: Diagnosis, Gastrointestinal stromal tumor, GIST, Prognostic factor
Gastrointestinal stromal tumors (GISTs) are rare cancers,
accounting for 0.1 % to 3.0 % of all gastrointestinal neo-
plasms . In adults, GISTs frequently occur in the stomach
(approximately 60 %) and 30 % of them appear in the small
intestine. On very rare occasions, GISTs originate from
the mesentery, omentum or retroperitoneum outside
the gastrointestinal tract [2,3].
GISTs range from incidental lesions a few millimeters
in diameter to large masses dozens of centimeters in size
and they have a broad range of presentations. Some are
identified clinically because they cause symptoms; but
most GISTs are asymptomatic and discovered inciden-
tally . A large number of new studies on histological
diagnostic criteria, GIST molecular biology and patho-
genesis, imaging strategies and surgical and adjuvant
treatment have been published [5,6] and consequently
more aspects of GISTs have been revealed and understood.
In spite of this, in some cases it is still difficult to diagnose
In this report, we describe a rare case of a huge GIST
of the stomach that had traversed the upper abdomen.
To the best of our knowledge, this is the largest tumor
reported in the literature. The tumor was completely
resected and our department of pathology characterized
it through immunohistochemistry (using, for example,
CD117, CD34, Dog-1) to evaluate the direction in which
it was tending to differentiate.
Our hepatobiliary surgery outpatient department
accepted a 66-year-old woman, who presented with a
year-long history of aggravating abdominal discomfort that
was mainly associated with distension and symptoms such
as epigastric fullness, eructation, decrease of food intake
and early satiety. She did not report any loss in body
weight. A physical examination revealed a palpable mass
in the upper abdomen, extending from the right hypo-
chondrial region to the left hypochondrial region, with
ordinary consistency and a smooth surface. Her liver and
spleen were not palpable distance from the costal margin,
and were not tender on palpation. All routine blood and
biochemical markers were normal. The levels of tumor
* Correspondence: email@example.com
Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of
Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
© 2012 Zhou et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative
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reproduction in any medium, provided the original work is properly cited.
Zhou et al. World Journal of Surgical Oncology 2012, 10:66
WORLD JOURNAL OF
markers assessed, including carbohydrate antigen 125 and
carcinoembryonic antigen, were within the normal ranges.
An upper gastrointestinal barium series revealed the
antrum, fundus and body of her stomach, as well as the
jejunum, were prominently compressed and infraplace-
ment (Figure 1). However, the gastroscope examination
did not show any abnormality. An abdominal ultrasound
revealed a sharply defined mass with an intact capsule
and opaque dark areas of fluid detected in the middle
region, suggestive of a huge cystic solid myoma. We were
not able to identify any connection between the adjacent
organs and the mass because the tumor was too large to
obtain a clear view.
Enhanced abdominal computed tomography (CT)
scans demonstrated a deformed liver of abnormal size
and structure, and a large low-density region featuring a
well-circumscribed border that overlay the entire left
liver and partially overlay the right liver. The tumor con-
tained inhomogeneous cystic component s mixed with
solid elements (Figure 2). In the arterial phase, complete
filling of the tumor with contrast material was never
observed; patchy enhancement indicated possible tumor
necrosis in the low-attenuation areas. The area of lower
density also did not enhance completely on delayed
scans. There were no signs of lymphadenopathy or liver
or pancreatic disease.
CT angiography (CTA) showed that the tumor was sup-
plied mainly by branches from the left and right gastric
arteries. The common hepatic artery, left hepatic artery,
splenic artery, superior mesenteric artery and left renal
artery next to the tumor were compressed. The portal vein,
inferior vena cava, left hepatic vein and superior mesenteric
vein were also compressed (Figure 3).
An exploratory laparotomy under general anesthesia
was performed and revealed a huge, thick-walled tumor
that almost filled the abdominal cavity. It appeared to pro-
trude from the anterior wall of the fundus of her stomach,
on the lesser curvature. The tumor was well-demarcated
from the surrounding organs (liver, spleen, transverse
colon), which were displaced but not involved with the
tumor. Furthermore, the tumor had no relationship with
the adjacent major vessels. In order to completely extirpate
the tumor, the proximal stomach and lower esophagus
were segmentally resected, and then an esophagogastrost-
omy was performed. No evidence of liver metastasis,
lymphadenopathy or peritoneal meta sta sis was found
(Figure 4). The tumor measured approximately 21 × 34 ×
11 cm in diameter and weighed 5.5 kg.
Figure 1 Upper gastrointestinal barium. (A) The fundus and body of the stomach are narrowly compressed and a high-density area can be
seen in the left-middle abdomen. (B) The antrum of the stomach and jejunum are also compressed and infraplacement.
Figure 2 Enhanced abdominal computed tomography shows a
large low-density area over the entirety of the left and part of
the right liver.
Zhou et al. World Journal of Surgical Oncology 2012, 10:66 Page 2 of 5
Histopathological examination of the resected specimen
revealed a stromal cell neoplasm with necrotic and
hemorrhagic areas, as well as a proliferation of spindle cells
with a mitotic count of less than five mitoses per 50 high-
Immunohistochemical analysis revealed the specimen to
be CD177 positive, CD34 positive, Dog-1positive, Ki-67
positive (1 %) and S-100 negative. The postoperative
course was uneventful and treatment with imatinib mesi-
late was initiated immediately. Our patient was discharged
20 days after surgery and advised to attend follow-up CT
scans of her abdomen in regular three to six months
GISTs are soft tissue sarcomas. They represent the most
common mesenchymal neoplasms of the ga strointestinal
tract. Based on an analysis of GIST histology, most of
them can be sorted into three major histologic patterns:
spindle cell type (70 %), predominantly epithelioid cell
type (20 %), or a mixture of both spindle and epithelioid
The immunohistochemical features of GISTs are used to
confirm diagnosis. Studies have confirmed that approxi-
mately 95 % of GISTs are positive for KIT (also known as
CD117), which plays a central role in pathogenesis. Gain-
of-function mutations in KIT lead to constitutive overex-
pression and autophosphorylation of c-kit, which induces
cells toward proliferation and/or away from apoptotic
pathways . However, many non-GISTs are also positive
for KIT, and about 5 % of GISTs have no detectable KIT
Many new gene mutations are used in identifying KIT-
negative GISTs or to differentiate GISTs from similar
tumors. About 80 % of KIT-negative GISTs were found
to have a platelet-derived growth factor receptor alpha
Figure 3 Computed tomography angiography. (A) The image illustrates a huge mass measuring about 30 cm in the maximum diameter. The
left hepatic vein (black arrow) is compressed to the right by the huge mass. In the second hepatic portal, the inferior vena cava (white arrow) is
also compressed. (B) The portal vein (black arrow) and superior mesenteric vein (red arrow) are compressed to the right. The trace of the splenic
vein (white arrow) is not clear. (C) The superior mesenteric vein (black arrow) is compressed. (D) The tumor is supplied mainly by branches from
the left (red arrow) and right gastric artery, although origin from the common hepatic artery is also possible. The common hepatic artery (blue
arrow), left hepatic artery and splenic artery (white arrow) are adjacent to the tumor. (E) In addition to the arteries mentioned in (C), the superior
mesenteric artery (black arrow) and left renal artery are also next to the tumor and compressed.
Zhou et al. World Journal of Surgical Oncology 2012, 10:66 Page 3 of 5
gene (PDGFRA) mutation, which results in an epithelioid
morphology. This discovery had been used in discrim-
inating between KIT-negative GISTs and other ga stro-
intestinal mesenchymal lesions [11,12]. BRAF mutations
have been revealed in a small number of high-risk GISTs
which develop from intestine lacking KIT or PDGFR A
mutations . A promising calcium-dependent and
receptor- activated chloride channel protein, known a s
DOG1, ha s emerged as a sensitive and specific marker
used in the setting of KIT-negative GISTs . Protein
kinase C theta, a downstream effector in the KIT signaling
pathway, is used to discriminate between GISTs and leio-
myosarcoma or other tumors which have similar histo-
pathology to GISTs . Recently, a novel biomarker
called carbonic anhydrase II, which can promote tumor
growth by contributing to intracellular alkalization and
extracelluar acidification, has been demonstrated to be
quite selective to GISTs among mesenchymal tumors .
The clinical presentation of GISTs is erratic. About
70 % of patient s are symptomatic and GISTs are asso-
ciated with a broad range of presentations, including
early satiety, bloating and some form of gastrointestinal
bleeding, either acute or chronic . However, approxi-
mately 30 % are a symptomatic or the GIST is detected
incidentally at autopsy. There is no physical finding that
specifically suggests the presence of a GIST. Although
there are several diagnostic modalities available, such as
barium examination of the gastrointestinal tract, CT or
abdominal ultrasound, none of them can confirm the
diagnosis. On some occasions, these examinations prove
to be deceptive with regards to tumor identification. As
in our case, CT images and an upper gastrointestinal
barium series suggested that the tumor possibly origi-
nated from the liver or gastrointestinal tract. In addition,
the abdominal ultrasound indicated the possibility that
the tumor came from the retroperitoneal organs. We could
not determine the diagnosis because signs were evident for
both possible diagnoses. Under these circumstances, we
decided to perform an exploratory laparotomy. In order to
explicitly determine the relationship between important
vessels, neighboring organs and the mass, CTA examination
was performed before the operation.
Because GISTs are usually radioresistant and insensitive
to chemotherapeutic agents, surgery remains the main
therapy for patients with primary GISTs who have no evi-
dence of metastasis. The tumor in our report was so huge
that it almost filled the abdominal cavity, approximately
21 × 34 × 11 cm. Although no such advanced case had
been previously reported in the literature, we were able to
completely remove the tumor with the proximal stomach
and lower esophagus.
There are also many GISTs which have metastases or
are unresectable using current technology. Imatinib
mesilate, which is an inhibitor of a family of structurally
related tyrosine kinase signaling enzymes, is currently the
most effective treatment for GISTs . However, some
patients are insensitive to imatinib mesilate, as almost im-
mediately after initiation or disease stabilization they then
experience disease progression while on medication. In
this situation, a multi-kinase inhibitor which inhibits, for
example, KIT, PDGFR and vascular endothelial growth
factor receptors 1 to 3, such as sunitinib, is indicated for
those who fail imatinib treatment . There are a few
patients with GISTs who fail both imatinib and sunitinib
treatment. In these cases, research has shown that nilotinib,
which specifically inhibits KIT, PDGFRA and BCR-ABL,
resolves the problem .
There is general agreement that tumor size and mitotic
count are the most important prognostic factors in
GISTs. These two features were the foundation for a
consensus approach to risk stratification of GISTs pub-
lished in 2002 . Subsequently, the criteria for the risk
stratification of GISTs have constantly been expanded
due to the availability of long-term clinical follow-up.
Based on the long-term follow-up of more than 1,600
patients, Miettinen and Lasota  suggested guidelines
for the risk stratification of primary GISTs based on mi-
totic index, size and site. Moreover, other pathologic
features , including cellularity, mucosal ulceration, and
the presence or absence of KIT or PDGFRA mutations,
have been clinically evaluated .
We have described an uncommon huge gastric GIST.
According to fundamental surgical principles in the
Figure 4 The tumor protrudes from the anterior wall of the
fundus, on the lesser curvature of the stomach, measuring 21 ×
34 × 11 cm in diameter and weighing 5.5 kg.
Zhou et al. World Journal of Surgical Oncology 2012, 10:66 Page 4 of 5
management of gastric GISTs, we completely resected the
tumor with the proximal stomach and lower esophagus.
The patient has been followed-up after the operation. Fur-
thermore, we will periodically examine the patient and fol-
low support guidelines for medical therapy. We hope the
presentation of this rare case could benefit others when
they encounter a similar diagnostic problems.
Written informed consent was obtained from the patient
for publication of this case report and any accompanying
The authors declare that they have no competing interests.
CL designed the study and LZ drafted the manuscript. CL is the guarantor.
All authors contributed to the intellectual context and read and approved
the final version.
Received: 19 December 2011 Accepted: 27 April 2012
Published: 27 April 2012
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Cite this article as: Zhou et al.: A rare giant gastrointestinal stromal
tumor of the stomach traversing the upper abdomen: a case report and
literature review. World Journal of Surgical Oncology 2012 10:66.
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