The Ileal Gastrointestinal Stromal Tumor: A Rare Site of Occurrence
Ahsan
Zil-E-Ali1*, Ali Akram Khan2, Syeda Naqvi3
1Departments of Surgery, Fatima Memorial Hospital, Lahore, Pakistan
2Internal
Medicine, Detroit Medical Center, Wayne State University, Michigan, USA
3Medicine, Jinnah
Sindh Medical University, Karachi, Pakistan
*Corresponding author: Ahsan Zil E Ali, Department of Surgery, Fatima Memorial Hospital, Lahore, 725 Shadman Rd, Lahore 54000, Pakistan. Tel: +9242111555600; Email: ahsanzileali@gmail.com
Received Date: 05 August, 2017; Accepted Date: 28 August, 2017; Published Date: 04 September, 2017
Citation: Ali AZE, Khan AA, Naqvi S (2017) The Ileal Gastrointestinal Stromal Tumor: A Rare Site of Occurrence. J Surg 2017: 160. DOI: 10.29011/2575-9760.000160
1. Abstract
Gastrointestinal Stromal (GIST) tumors
are one of the common malignancies arising from mesenchymal tissue. They can be
present anywhere in the gastrointestinal tract, most commonly in the stomach and
small intestine; less frequent in colon, rectum, and esophagus. We describe a
rare presentation of GIST tumor in Ileum in a young patient without any risk
factor and positive family history. Furthermore, this patient responded well to
surgical resection and Imatinib therapy. Due to high recurrence rate, five
years follow up with annual whole body Computed Tomography scan (CT) was
advised.
1.
Introduction
GIST tumor was the term first coined by Mazur and Clark in 1983.They originate from Cajal cells, which are pacemakers for gut peristalsis, present in myenteric plexus of the gastrointestinal tract. In 1998, mutation of KIT was recognized as one of the causes of GIST tumors. In 2001, selective inhibitors of KIT and tyrosine kinase like Imatinib were introduced[1].Most GIST tumors are sporadic in occurrence, rarely also associated with syndromes like Neurofibromatosis, Familial GIST, Carney’s Triad and Carney-Stratakis syndrome. The common types of GIST are spindle, epithelioid and mixed. The immunohistochemical stain like KIT (CD117) is a sensitive marker for the diagnosis[2]. Mostly GIST tumors, if symptomatic presents with gastrointestinal bleeding as the most common symptom. Tumors less than 6 cm tends to remain asymptomatic or an incidental finding. If symptomatic management options are surgical resection of the tumor. In addition to that pre-operative Imatinib can also be given to enhancing tumor shrinkage before surgery. After surgery, the recurrence rate is around 70% in 5 years and one can predict a higher chance of recurrence by high mitotic activity (can be predicted by Ki-67)[1,2].
2. Case Report
A 38 years old man presented with right hypochondriac pain with nausea for past 6 months and multiple vomiting episodes during this time, along with dark stool on some occasions and early morning abdominal tenderness. The patient had fatigue and malaise along with the gastrointestinal discomfort. Patient’s family history was positive for colorectal and breast cancer. He is a smoker, one pack per day for last five years.On physical examination, he had pale conjunctivae and signs of anemia. On deep palpation of the abdomen, no major gross abnormality was appreciated. His baselines including Complete Blood Count, Liver function test and Gastrointestinal series were ordered. Those were significant for anemia and a pelvic mass. A computerized tomography was ordered to confirm the diagnosis and it showed a huge mass. A surgical consult was offered and surgical removal of the mass was proposed as the management option.The patient consented to the procedure and general anesthesia. A routine pre-operative protocol for blood workup was carried, which was normal. ‘Battle’ incision was planned and the lower GI tract was explored for the mass. The mass was arising from ileal loops abutting the sigmoid colon in its plane and compressing the dome of the bladder. The mass was highly angiogenic, with many vessels visible on gross presentation. A careful surgical resection from the ileal wall was done. The mass was sent for the histopathological analysis that showed a gastrointestinal stromal tumor on hematoxylin and eosin stain with c-kit/CD117 positive on immunohistochemistry. An oncologist was involved and imatinib was given as a pharmacological treatment to prevent recurrence.The occurrence at the aforementioned age and the site located, makes it a peculiar and different than the classical presentation. It is also notable that the mass revealed on CT image could have led to other gross abnormalities, which were absent.
Up to two-thirds of patients with GISTs will have metastasis
or recurrence. The median time for recurrence is 19 to 25 months; however,
follow up for recurrence is long term as metastasis have been reported to occur
as late as 30 years after the removal of the primary tumor.2 The
overall relative 5-year survival rate of people diagnosed with a malignant GIST
was estimated to be about 76%. In the case where the tumor was confined to the
organ where it started, the 5-year relative survival was 91%. When the same
tumor spread into nearby tissue (and/or lymph nodes) the survival rate went
down to 74 % while with distant sites the 5-year survival rate was 48%[5,6].
The relevant Kaplan-Meier curves show that the 1) recurrence-free survival and 2) the overall survival, both are shorter for patients with gastrointestinal bleeding caused by GIST. Gastrointestinal bleeding is an independent risk factor for GIST recurrence and death of the patients and should be considered a significant indicator of poor prognosis[8]. it is wise to discuss the possibility and viability of robot-assissted surgeries which have gained enormous acceptance among surgeons. The reason of their popularity is that they provide highly magnified three-dimensional image view that improves hand-eye coordination. Such systems provide beneficial effects including those in the range of motion scaling, multi-dimensional stereoscopic visualization, excellent instrument dexterity with tremor fibrillation which ensures accuracy of precise movements in tight spaces. This advent not only solves many problems regarding surgical expertise but also overcomes human error while providing better precision. However, a study performed in Italy brought forth some limitations of the procedure where robot-assisted surgeries did not readily manage cases of minimal bleeding. Although there were no fatalities, it did contribute to the morbidity. It has been established that to shift the learning curve to master, 10 solo surgeries must be performed alongside a fellow robot. The factors limiting the success rate of this expensive procedure include limited arms on the robot, lesser popular academic training as well as large diameters of the instrument[9,10].
It is recommended that a thorough evaluation is
needed with right diagnostic tests and more importantly the appropriate
intervention. Keeping in mind the prognostication, one must keep imatinib and
other similar chemotherapies in point of discussion while explaining the disease to the patient(Figure 1-4).
Figure 1: Intra-operative visualization of GIST tumor from the ileum
with high vasculature visible.
Figure 2: Intra-operative handling of the tumor within the ileal
loops.
Figure 3: CT scan image of the ileal GIST tumor
compressing the adjacent tissue and protruding into pelvic area.
Figure 4:Histopathological picture showing
whorled pattern and high N:C ratio cells.
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