Department of Gastrointestinal, Hepatobilliary & Pancreatic Disorders, Bangladesh Institute of Research and Rehabilitation for Diabetes, Endocrine and Metabolic Disorders (BIRDEM), Shahbag, Dhaka, Bangladesh
Department of Surgery, Bangladesh Institute of Research and Rehabilitation for Diabetes, Endocrine and Metabolic Disorders (BIRDEM), Shahbag, Dhaka, Bangladesh
Department of Gastroenterology, BRB Gastroliver Hospital, Dhaka, Bangladesh
objectives:Gastrointestinal stromal tumor (GISTs) is an uncommon and rare
disease in Bangladesh. Our aims were to describe
socio-demographic characteristics, clinical presentations, anatomical location,
morphological variation, treatment and outcome of GIST in ten cases.
Methods:This study included consecutive ten cases of GISTs diagnosed
and treated in two tertiary level hospitals in Dhaka, Bangladesh from 2013 to
2016. Patients’ socio-demographic characteristics, clinical presentations,
anatomical location, histological types, presence of CD117 markers were
determined. Outcome of the treatment by surgical intervention and imatinib mesylate
(400mg/day) were evalauted.
Results: Total 10 patients were included in the study. Among them 6 were
male and 4 were female. The age range was 32-74 years. Abdominal pain, haematemesis,
melaena, haematochezia and anaemia
were the most common presentation. One patient had dysphagia and another had
features of subacute intestinal obstruction. Five patients had GIST in the
stomach (50%), two had in colon and one in esophagus, duodenum and ileum
respectively. CD 117 was positive in 8 cases, majority had spindly type cell
with low mitotic figure. Imatinib therapy was given in all the cases except two
patients. Disease recurrence in the form liver metastasis was found in two
cases and both died. Disease free survival for more than 2 years was observed
in 4 cases.
Conclusion: Haematemesis and melaena were common presentation of GISTs.
Stomach was the most common site for GISTs and majority had spindle type of
cells and positive CD117 marker. Surgical intervention and imatinib therapy was
IMC J Med
Sci 2017; 11(2): 45-49
Address for Correspondence: Dr. Md. Nazmul Hoque,
Consultant and Head, Department of GHPD, Ibrahim Medical College & BIRDEM
General Hospital, 122 Kazi Nazrul Islam Avenue, Dhaka, Bangladesh. Email: firstname.lastname@example.org
stromal tumors (GISTs) are the mesenchymal tumors of the gastrointestinal tract
(GI) and account for less than 1% of GI
tumors . About 60%-70% GIST arises
from the stomach and the rest are from small
intestine (20% to 30%), colon and rectum (5%), and esophagus (<5%) .
A small number of stromal tumors may originate from outside the
gastrointestinal tract. These are designated as extragastrointestinal stromal
tumors (EGISTs) .
lesions were classified as leiomyomas or leiomyosarcomas because they possessed smooth muscle features. With the
advent of immunohistochemical staining techniques GISTs now are recognized as a
distinct group of mesenchymal tumors. GISTs express c-kit protein also known as
CD117, and is considered a specific marker that differentiates GIST from other
mesenchymal tumors such as leiomyomas .
The most common
clinical manifestation for symptomatic GIST is pain in abdomen and GI bleeding
from mucosal ulceration . Other presentations include mass in the
abdomen or intestinal obstruction. Endoscopy and colonoscopy clearly delineate
the site and macroscopic appearance. However, ultrasonography (USG) and
computerized tomographic (CT) scan are useful to determine the location of tumor
in the gastric or intestinal wall .
Tumors less than 2 cm in diameter with a mitotic rate of
<5/50 HPF (high power field) have been shown to have lower risk of recurrence
than larger tumors. All GIST tumors should be considered to have malignant
potential . At present, surgery remains the mainstay of treatment for GIST. Recently,
imatinib mesylate has been introduced as an adjunct therapy for metastatic
disease, for non resectable tumor and for prevention of recurrence . In this report, we describe the
socio-demographic characteristics, clinical presentations, anatomical location,
investigations, morphological variation, treatment and outcome of GIST
encountered over four years period at two tertiary care hospitals.
the patients diagnosed with GISTs from 2013 to 2016 at two tertiary care of
hospitals (BIRDEM General Hospital and Gastroliver Hospital) of Dhaka city were
included in the study.GIST
cases were analyzed for age, sex, clinical features, tumor location, size,
histological characteristics and CD117 marker. Types of surgery, post operative
event, outcome of imatinib therapy, disease recurrence, recurrence site and
survival rate were also analyzed. Mitotic figure defined as number of mitosis
per 50 HPF (high power field). Disease recurrence means local recurrence or
distant metastasis. GIST cases with inconclusive histological findings during
the study period were excluded. Long term follow up at intervals were done by
clinical examination, routine blood test and abdominal USG.
total of ten cases of GIST were included in the study for analysis. Six of them
were male, 4 were female. Their age ranged from 32-74 years. Five patients
presented with GI bleeding in the form of haematemesis, melaena and
haematochezia, three patients had abdominal pain, two patients had anemia, one
patient had dysphagia and one had features of subacute intestinal obstruction (Table
of ten cases, 5 (50%) tumors were present in the stomach. Among stomach GIST,
two involved the fundus, two were in antrum and one in mid body near the
greater curvature. Two patients had GIST in the caecum. Detail distribution of
GISTs is shown in Table-1. Partial gastrectomy and gastrojejunostomy (Billroth II) were
done in three cases and fundectomy in two cases by using stapler. Right
hemicolectomy and ileo transverse anastomosis was done in two cases. In case of
ileal GIST, resection and anastomosis was done and duodenotomy and enucleation
was done to manage the duodenal GIST (Table 1). Only two patients experienced
diarrhea during post operative period.
Average sizes of the GIST were 4.2x4.8 cm.
Histological examination revealed spindle type of cell in 80% cases and 20 % cases
had mixed type of cell (Table 2). CD117 was positive in 80% cases, two patients
were negative for CD117 but histologically both were diagnosed as GIST. Average
mitotic figure was 1.4/50 HPF. Mitotic figure were absent in three cases. All
patients were advised imatinib therapy. Eight out of ten patients took imatinib
for 6 months to 3 years (Table 2). Disease recurrence in the form of liver
metastasis was found in two cases despite of taking imatinib for more than one
year. These two patients had large size GIST and mitotic figures were more than
that of other cases and both expired during this follow up time due to liver
metastasis or recurrence was observed in four cases after 2-3 years of follow
up. No metastasis or recurrence was observed in four cases after 2-3 years of
follow up. Detail recurrence and disease status of all the cases in follow up
after a range of period (6 month to 3 years) are shown in Table-2.
Table-1: Salient clinical features, anatomical sites of
GISTs and types of surgical intervention (n=10)
Table-2: Histological types, duration of imatinib
therapy and recurrence rate of diseases among the study population
is a very uncommon and rare disease in Bangladesh. Few sporadic case reports of
GIST among Bangladeshi patients were published in the literature . GISTs are most common in adults between 50-60 years of age .
However, a case of jejunal GIST in a 38 year male patient has also been
reported . In our series, one patient had esophageal GIST aged 32 years and presented
with dysphagia. The oldest case in these series was a 74 years lady. The common presenting feature
in this study were GI bleeding. Gross GI bleeding was present in 50% cases and
20% cases had occult bleeding. Abdominal pain, abdominal distension, abdominal
lump and dysphagia were also present. Similar presentations were described in
other studies [10,11].
The vast majority of
GISTs (60 to70%) arise in the stomach, with 20-30% originating in the small
intestine and the remainder 10% occurring in the esophagus, colon and rectum .
series, stomach was involved in 50% cases and 20% were in the colon. Most of the GISTs were small and asymptomatic and were
discovered incidentally during evaluation for unrelated problems. When the
lesion grows over 2 cm in size, ulceration may occur and symptoms like
epigastric pain and gastrointestinal bleeding become more common at that time.
Spontaneous rupture of large mass into gastric lumen has also been reported .
The average size of the lesion in our cases was 4.2x4.8cm.
GIST’s may be spindle or epithelioid type. Spindle cell GISTS are more common.
Histologically, most cases fall into one of the following three categories, spindle
cell type -70%, epitheliod type - 20%, mixed type -10% . In our
series, 80% cases were spindle type; remaining 20% were defined as mixed
variety. In general, GISTs with less than 1 mitotic figure per 50 HPF are
correlated with benign behavior. It has been reported that presence of 1-5 mitotic
fegures per 10 HPFs suggest potential malignancy. A finding of more than 5 mitotic
figures per 10 HPFs indicates malignancy. A finding of more than 10 per 10 HPFs
denotes high-grade malignancy .
About 10% GISTs
exhibit malignant behavior . Prognostic factors include age, location
staging, tumor size and mitotic activity. Esophageal and gastric GISTs have a
better prognosis than intestinal GISTs. Size and mitosis are most useful
predictors. In our series, the average mitotic figure was 1.4/50 HPF, and disease
recurrence were found in two cases. These two patients had large tumor size and
high mitotic figure (3/HPF).
In our study, 80% of
tumors were positive for CD 117 marker. CD117 positivity (diffuse cytoplasmic staining
with membranous accentuation) is seen nearly in all GISTs with spindle cell or
epitheloid morphology, though less intensely in the latter. A small number of
otherwise typical GISTs may be CD117 negative and immune-reactivity for CD117
is sometimes lost in metastatic cells .
Surgery, in the form
of wide surgical resection, is the mainstay of therapy for non-metastatic
GISTs. Laparoscopic surgery has been shown to be effective for removal of these
tumors without the need of large incisions . But in our series, all patient
was first approved by FDA in 2001 . It is the first and only effective drug
for treatment of GIST at present. According to the latest ASCO guidelines,
recurrence free survival is increased in patients taking imatinib 400 mg/day
for one year of . In our series, 2 patients did not receive imatinib but
remained disease free during the follow up period. Disease recurrence was found
in two cases even after taking imatinib.
In our study, common presentation of GIST was
gastrointestinal bleeding and the common site of involvement was stomach. Histologically majority were positive for spindle
type of cell and CD117 marker. Surgical intervention along with imatinib
therapy was effective for disease free survival in most of the cases.
Conflict of interest: None declared.
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