REVIEW

CURRENT
OPINION Gastrointestinal stromal tumor: epidemiology,
diagnosis, and treatment
George Mantese

Purpose of review
The purpose of this review is to review the past year’s literature to provide comprehensive information to
researchers, physicians, and the general public regarding the epidemiology, diagnosis, and treatment of
gastrointestinal stromal tumors (GISTs). Common ground as well as divergent viewpoints will be highlighted
and discussed.
Recent findings
The diagnosis of GISTs may involve imaging tests such as computed tomorgraphy scan and MRI,
endoscopy with or without endoscopic ultrasound, and biopsy. Only biopsy, however, can yield a positive
diagnosis. As most GISTs express KIT protein, immunostaining for KIT and/or molecular genetic testing for
mutations in KIT can diagnose 95% of GISTs. Regorafenib, a drug that inhibits various protein genes that
lead to GIST development is a relatively new treatment modality.
Summary
The current review should enable clinicians to best select the diagnostic and treatment approaches to GIST.
Keywords
gastrointestinal stromal tumor, KIT gene, rogorafenib, review

INTRODUCTION growth and proliferation of GIST cells. A positive

Gastrointestinal stromal tumors (GISTs) are rare
sarcomas of the soft tissue that can occur anywhere
along the gastrointestinal tract. The most common
site is the stomach followed by the small intestine,
colon, and rectum. GISTs were originally misclassi-
fied as leiomyomas, leiomyosarcomas, and schwan-
nomas. Ultrastructural, immunohistochemical, and
molecular biological techniques have allowed us to
now recognize that GISTs originate from interstitial
cells of Cajal (ICC) or a common precursor cell. ICCs
are present throughout the gastrointestinal tract
where they function as pacemaker cells to coordi-
nate peristalsis. GISTs develop through oncogenic
gain of function mutations in KIT or platelet-derived
growth factor receptor (PDGFR) genes that lead to
constitutive activation of the tyrosine kinase recep-
tor [1]. Receptor tyrosine kinase physiologically
regulate growth and proliferation. Oncogenic muta-
tions in these receptors account for about one-third
of human malignancies. More recently, ETV1 [E
twenty-six (ETS) variant 1], a member of the ETS
family of transcription factors, has been shown to be
highly expressed in ICCs and overexpressed in
GISTs. ETS proteins regulate many target genes
involved in cell growth, proliferation, and differen-
tiation. ETV1 has been shown to be required for the
feedback loops exists in GISTs whereby constitu-
tively activated KIT receptor tyrosine kinase enables
target gene binding of ETV1 which, in turn, directly
enhances KIT expression. ETV1 may be a future
therapeutic target for treating GISTs.
Risk factors for development of GIST include
inherited familial GIST syndrome and primary
familial GIST syndrome. Tanaka et al. [2] argue that
familial risk is associated with primary biliary chol-
angitis. GISTs may be classified according to size,
location, and mitotic index. Symptoms include
abdominal pain, nausea, and bleeding [3]. This
review will focus on the epidemiology, differential
diagnosis, and treatment of GIST.
INCIDENCE AND DISTRIBUTION
As most GISTs are asymptomatic, they are not
commonly recognized during life. Consequently,
worldwide incidence of GIST is estimated at one
Secretaria de Saúde de Porto Alegre, Porto Alegre, Brazil
Correspondence to George Mantese, Secretaria de Saúde de Porto
Alegre, Rua Almirante Barroso, 720/307, Porto Alegre 90020-220,
Brazil. Tel: +55 51 997511290; e-mail: georgemantese@hotmail.com
Curr Opin Gastroenterol 2019, 35:555–559
DOI:10.1097/MOG.0000000000000584

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Stomach and duodenum

and may present with abdominal pain and/or gas-

KEY POINTS
 GISTs are rare but increasingly recognized tumors of
the gastrointestinal tract.
 Diagnosis is by disease and involves immunostaining of
tissues for KIT, DOG-1, and CD34 proteins.
 Prognostic factors include tumor site, size, mitotic
index, location, and spread.
 Surgery is the primary curative treatment but
recurrences do occur.
 Adjuvant chemotherapy includes the tyrosine kinase
signaling inhibitors imatinib, sunitimib, and
regorafenib. However, these medications are unlikely
to be beneficial in patients lacking a mutation in KIT or
platelet-derived growth factor receptor as well as in
patients with a mutation in platelet-derived growth
factor receptor D842V.
to two per 100 000 and prevalence of 13 people per
&& && & &
100 000 [4,5 ,6,7 ,8 ,9 ]. Autopsy studies, how-
ever, suggest that small (<1 cm) GISTs may be iden-
tified in 25% of unselected individuals when the
gastrointestinal tract is carefully scrutinized. GISTs
occur at any age, but are most commonly diagnosed
&&
in those older than 60 [5 ]. Men and woman are
&
equally affected [8 ]. Approximately 60% of GISTs
occur in the stomach and 30% in the small intes-
tines [4,6]. The median tumor size at diagnosis is
6 cm. From 2001 to 2015, the incidence of GIST
has increased, possibly due to increased apprecia-
tion as well as the incidental discovery of small
GISTs during routine upper endoscopy and capsule
&
endoscopy [8 ].
trointestinal bleeding [11,12]. Bleeding may occur
into the bowel or abdominal cavity. Approximately
20% of patients have metastasis at time of diagnosis.
The most common sites for metastases are the liver,
abdominal cavity, and lymph nodes. Less common
presentations include nausea, pleuritic chest pain,
and pelvic pain as well as small bowel obstruction.
Most GISTs are sporadic (>97%). Familial GISTs are
rare (neurofibromatosis type 1, Carney–Stratakis
syndrome, and Carney triad) but present differently,
often with hyperpigmentation, urticaria pigmen-
tosa, and an increase in nevi [11].
DIAGNOSIS
Pathological diagnosis
GISTs are usually diagnosed using immunohis-
tochemistry directed to their expression of KIT pro-
tein (95%), a receptor tyrosine kinase protein, also
known as stem-cell growth factor receptor or
CD117, that is expressed on ICCs. KIT is also
expressed on hematopoietic stem cells, mast cells,
melanocytes, and germ cells. The main oncogenic
drivers of GIST are activating mutations in KIT
(90%) and PDGFR(10%). A smaller subset of
GISTs (10%) arise from mutational inactivation
of neurofibromatosis 1 protein (NF1) or mutational
activation of RAS or BRAF. Some GISTs express CD34
(70%), a transmembrane phosphoglycoprotein first
identified on hematopoietic stem cells, but subse-
quently identified on highly proliferative multipo-
tent mesenchymal stromal cells [13]. Most GISTs do
not stain with smooth muscle markers such as des-
min, actin, or myosin [9 ,14–16].
&

Table 1 summarizes the various antibodies clini-

cians can use to diagnose GIST in various tissues.
CLINICAL PRESENTATION CD117 (90–95%), DOG1 (98%), and CD34 (70%)
GISTs are usually asymptomatic until they reach a are the most useful. DOG1 (discovered on GIST-1) is
size of 6 cm [10 ] Large GISTs are often vascular
&&
a gene that encodes the chloride channel protein

Table 1. Gastrointestinal stromal tumor pathological diagnosis summary

Antibody % Positive results in GIST Other tumors exhibiting positive immunoreactivity

CD117/c-Kit 90–95% Clear cell sarcoma, Melanoma, perivascular epithelioid cell tumor (PEComa)
CD34 80–85% for gastric and 50% f Solitary fibrous tumor, vascular tumor, spindle cell lipoma
or small intestinal GIST
PKC-theta 90% Desmoid, smooth muscle tumor, PNST
h-Caldesmon 60–80% Smooth muscle tumor
SMA 30–40% Myofibroblastic tumor
S-100 5% Melanoma, granular tumor, PNST
Desmin 1–2% Smooth muscle tumor

GIST, gastrointestinal stromal tumor; PNST, peripheral nerve sheath tumor.

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 Gastrointestinal stromal tumor Mantese

anoctamin-1 and is independent of KIT or PDGFR
mutation status. In rare cases, genotyping can be
used to identify GISTs [17,18]. Examples include
neurofibromatosis type 1 (mutated NF1), Carney–
Stratakis syndrome (GIST and paraganglioma due to
germline mutation in the succinate dehydrogenase
(SDH) mitochondrial tumor suppressor gene path-
way), and Carney triad (GIST, pulmonary chon-
droma, and extra-adrenal paraganglioma) due to
epigenetic hypermethylation in the SDH complex
genes. Carney–Stratakis syndrome is an autosomal
dominant inheritable disease whereas the Carney
triad is nonhereditary. SDH-deficient GISTs occur
almost exclusively in the stomach, exhibit a unique
growth pattern of nests of tumor cells separated by
septa of smooth muscle cells, and usually follow an
indolent course.
Diagnostic Imaging
Imaging modalities useful to diagnose GIST include
computerized acial tomorgraphy (CT scan), PET
&&
scan, MRI scan, and ultrasound [5 ,6,19,20]. CT
scan seems to provide the highest yield, especially
in small bowel GISTs, but radiation exposure may
be a concern in children and some adults [21].
GISTs above 5 cm typically appear exophytic and
hypervascular on CT scan whereas those below
5 cm are usually endoluminal polypoid masses
[18,22] CT scans have the added benefit of demon-
strating local invasion and metastasis [6]. PET
scans are useful as GISTS typically exhibit strong
[17] F-fluorodeoxyglucose uptake. On MRI, small
GISTs appear as round tumors with strong and
homogeneous arterial enhancement whereas
large GISTs appear as lobulated tumors with mild
heterogeneous gradual enhancement often with
intratumoral cystic change.
Prognostic factors, risk stratification, and
staging
Size, mitotic index, location, and rupture are the
four most important prognostic factors (Table 2)
[4,23,24]. The cutoff for size is 5 cm with those less
than 5 cm having the best prognosis. Because GISTs
are typically of low mitotic index, 50 high power
fields (HPFs; 5 square mm), rather than the more
typical 10 HPFs, are used to assess mitotic activity.
Other prognostic factors include postoperative ima-
tinib and curative resection recurrence.
According to Gyvyte et al. [25], GIST can be
categorized into four different stages based upon
the mitotic rate, size, spread to lymph nodes, and
distant metastasis. In general, the lower the stage,
the better the prognosis. Stage I tumors have a low
mitotic rate and no spread to lymph nodes. Stage IA
tumors are between 2 and 5 cm whereas stage IB
tumors are between 5 and 10 cm. Stage II tumors
have a high mitotic rate but are 5 cm or less and
without lymphatic spread [25,26]. Stage III tumors
have a high mitotic rate, no lymphatic spread, but
are greater than 5 cm in diameter. IIIA tumors are
between 5 and 10 cm whereas IIIB tumors are greater
&
than 10 cm [27 ]. Stage IV tumor can be of any size
or mitotic rate but have spread to lymph nodes or
&
distant sites such as the liver [28 ].
Treatment
Treatment is predicated upon size, location, and
spread [29]. The primary modality for localized GIST

Table 2. Gastrointestinal stromal tumors staging and risk assessment guidelines

Tumor parameters Risk for disease progression (%)

Mitotic index
Size (cm) Duodenum Rectum Gastric Ileum
Equal or greater than 5 per 50 HPF
2 None None None None
>2–5 8.3 Low 8.5 Low 1.9 Very low 4.3 Low
>5–10 No data No data 3.6 Low 24 Moderate
>10 34 High 75 High 10 Moderate 52 High
Less than 5 in every 50 HPF
2 No data 54 High None High
>2–5 50 High 52 High 16 Moderate 73 High
>5–10 No data No data 55 High 85 High
>10 86 High 71 High 86 High 90 High

HPF, high power field.

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Stomach and duodenum
is surgery and 60% of patients are cured [30].
Surgery may be used to remove a solitary tumor
or as secondary therapy after reduction in tumor
size by adjuvant chemotherapy. Caution should be
exercised to prevent rupturing the tumor capsule.
Surgery is generally not used for metastatic lesions as
these are prone to rupture with further spread [31].
Small tumors (<5 cm) can be managed laproscopi-
cally whereas larger tumors generally benefit from
& clinical symptomatic presentation is abdominal
open surgery to avoid rupture [4,30,32,33 ]. On the
contrary, 10% of GISTs recur postoperatively
[30,32]. Preoperative chemotherapy with imatinib,
a KIT (and PDGFR) inhibitor, can be considered to
shrink large tumors and limit surgical resections.
The precise duration is not known, but, for some
patients, imatinib has been given for up to
12 months to allow maximal tumor shrinkage. A
limitation of preoperative chemotherapy is that risk
stratification/estimation may be unreliable as tumor
mitotic activity is usually reduced. Imatinib works
best for GISTs with KIT exon 11 (90%) and 13
mutations (1%) and less well with KIT exon 9
(8%) mutations and PDGFR exon 12, 14, and 18
mutations. It has no or limited efficacy in patients
with the PDGFR exon 18 D842V (8%) mutation or
patients with GIST lacking KIT and PDGFR mutations.
Adjuvant chemotherapy of operative GISTs
includes the tyrosine kinase signaling inhibitors,
imatinib and sunitimib [30,32,34]. The response
rate (RR) to imatinib, 400 mg/day for 1–2 years, is
82% [18,35]. Some GISTs can be controlled for
many years with imatinib although many develop
resistance due to secondary KIT mutations. Patients
with KIT exon nine mutations benefit from a higher
dose of imatinib, usually 400 mg twice daily Imati-
nib acts on particular amino acid within the ATP and
activation loop and is suitable for use when the
tumor is more than 3 cm and near a vital organ
[36]. Imatinib has extended the median overall sur-
vival for patients with metastatic GIST from 1.5 to 5
years. Sunitinib acts on a different amino acid
within the ATP pocker and is typically used when
the response to imatinib wears off [30]. Esophageal
GISTs may respond better than those located in the
&&
stomach to the tyrosine kinase inhibitors [37 ].
Newer therapies
Regorafenib, a multikinase inhibitor active against
KIT and epidermal growth factor receptor as well a
number of other kinases, was approved in 2013
[38,39] Currently, it is used as a third-line medica-
tion after failure to imatinib and sunitinib; it offers
a 50% RR [4,40]. Postoperative radiation may yield
added value when combined with perioperative
chemotherapy, especially for single progressing liver
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&&
or intra-abdominal lesions [41 ]. Embolization and
radiofrequency ablation have also been used to treat
liver metastases.
CONCLUSION
In conclusion, the diagnosis and management of
GIST has evolved and continues to evolve. Although
most GISTs are asymptomatic, the most common
pain and/or bleeding definitive diagnosis is predi-
cated upon immunostaining of tissue, usually with
antibodies directed against KIT, DOG-1, and CD34.
Therapy is contingent upon accurate clinical stag-
ing. Validated risk stratification tools depend upon
tumor size, mitotic index, location, distant spread,
and rupture. The primary treatment modality is
surgery whereas tyrosine kinase inhibitors such as
imatinib, sunitinib, and regorafenib are adjunctive,
although they overall improve survival.
Acknowledgements
None.
Financial support and sponsorship
None.
Conflicts of interest
There are no conflicts of interest.
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