A case of giant unruptured jejunal gastrointestinal stromal tumor

Pandiaraja Jayabal; Shalini Arumugam

doi:10.4103/aort.aort_2_20

CASE REPORT

Year : 2021 | Volume : 1 | Issue : 1 | Page : 60-63

A case of giant unruptured jejunal gastrointestinal stromal tumor

Pandiaraja Jayabal¹, Shalini Arumugam²
¹ Department of General Surgery, Dr. Mehta Hospital, Chennai, Tamil Nadu, India
² Department of Community Medicine, ACS Medical College, Chennai, Tamil Nadu, India

Date of Submission	29-Dec-2020
Date of Decision	25-Jan-2021
Date of Acceptance	25-Apr-2021
Date of Web Publication	23-Jul-2021

Correspondence Address:
Dr. Pandiaraja Jayabal
26/1, Kaveri Street, Rajaji Nagar, Villivakkam, Chennai - 600 049, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aort.aort_2_20

Abstract

A gastrointestinal stromal tumor (GIST) is a most common mesenchymal tumor of the gastrointestinal tract. GIST is more common on the stomach, followed by the small intestine and rarely occur in other locations. We present an unusual case of large jejunal GIST arises from the antimesenteric border. Most of the small intestinal GIST present as a mobile mass in the right iliac fossa. Hence, a patient with mobile right iliac fossa mass without signs of obstruction or minimal signs of obstruction should consider the small bowel GIST as one of the differential diagnosis and plan for prompt intervention. Good preoperative imaging will diagnose small intestine GIST. Proper preoperative diagnosis and timely intervention will reduce the complication such as tumor rupture, spillage, R1 resection, and local recurrence. Postsurgical chemotherapy with Imatinib will reduce the recurrence rate and increase the survival.

Keywords: Gastrointestinal stromal tumor, jejunal gastrointestinal stromal tumor, right iliac fossa mass, unruptured

How to cite this article:
Jayabal P, Arumugam S. A case of giant unruptured jejunal gastrointestinal stromal tumor. Ann Oncol Res Ther 2021;1:60-3

How to cite this URL:
Jayabal P, Arumugam S. A case of giant unruptured jejunal gastrointestinal stromal tumor. Ann Oncol Res Ther [serial online] 2021 [cited 2022 May 23];1:60-3. Available from: http://www.aort.com/text.asp?2021/1/1/60/322145

Introduction

A gastrointestinal stromal tumor (GIST) is a most common mesenchymal tumor of the gastrointestinal tract. GIST is more common in the stomach, followed by the small intestine and rarely occur in other locations.^[1] The jejunum is one of the uncommon locations for GIST. The small intestine GIST is rarely symptomatic unless it grows large mass and produces compression symptoms or rupture into the peritoneal cavity. Most of the small intestinal GIST present as a mobile mass in the right iliac fossa. Hence, the patient with mobile right iliac fossa mass without signs of obstruction or minimal signs of obstruction should consider the small bowel GIST as one of the differential diagnosis and plan for prompt intervention. The early surgical intervention leads to Ro resection rather than R1 resection due to rupture and tumor spillage.

Case Report

A 57-year-old male presented with complaints of abdominal pain for 1-day duration. There is a history of nausea and vomiting for 1 day. There is no history of constipation, diarrhea, hematemesis, and bleeding per rectum. He is a known case of diabetes in irregular medication. He denies a history of loss of appetite and loss of weight. On examination, he is well-built and nourished. His vitals are blood pressure – 100/70 mmgH and pulse rate – 110/min. Per abdominal examination showed a mobile mass of 18 cm × 15 cm in the right iliac fossa.

His routine blood investigations are normal except increased blood sugar level – 360 mg/dl. Ultrasound examination of the abdomen showed a heterogeneous mass of 15 cm × 12 cm in the right iliac fossa. The contrast computed tomography (CT) showed a large well-defined mild-to-moderate heterogeneous intraperitoneal mass of 16.5 cm (craniocaudal) × 12.2 cm (anteroposterior) x 13.1 cm (width) [Figure 1]. The mass is arising from distal jejunum and jejunoileal junction [Figure 2]. Mesenteric fat stranding is visualized surrounding the lesion. There is no evidence of lymphadenopathy. On intravascular contrast administration, we can observe feeding vessels from jejunal branches of the superior mesenteric artery [Figure 3].

Figure 1: Contrast computed tomography of the abdomen with sagittal view showed large well-defined mild to the moderate heterogeneous intraperitoneal mass of 16.5 cms (craniocaudal) × 12.2 cms (anteroposterior) × 13.1 cm

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Figure 2: Contrast computed tomography of the abdomen with coronal view showed mass has arisen from distal jejunum and jejunoileal junction

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Figure 3: Contrast computed tomography of the abdomen with a sagittal view showed feeding vessels from jejunal branches of superior mesenteric artery

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The patient plans for immediate surgical intervention. He underwent laparotomy under general anesthesia. The intraoperative picture showed a mass of 20 cm × 15 cm × 12 cm on the antimesenteric border of jejunum, around 60 cm from the DJ flexure and the mass is about to rupture [Figure 4]. Segmental resection of the small bowel GIST is done with an adequate margin [Figure 5]. On the cut surface, the tumor is fairly encapsulated and showed grayish white, nodular areas with a variegated appearance showing necrosis, cystic changes, hemorrhage, mucoidal and myxoid changes, respectively. Postoperative histopathology showed spindle-shaped cells with elongated nuclei and eosinophilic cytoplasm [Figure 6]. The overlying jejunal mucosa showed focal mucosal ulcerations. Immunohistochemistry of the tumor showed it is positive for C-KIT and CD 34 and negative for smooth muscle actin, muscle-specific actin, S100, and desmin. Ki-67 proliferative index is 0.8%. The patient is categorized as high-risk GIST due to high mitotic index and large size of the mass. The patient is on T. Imatinib 400 mg once daily for the past 2 years.

Figure 4: Intraoperative picture showed a mass of 20 cm × 15 cm × 12 cm on the antimesenteric border of jejunum, around 60 cm from the DJ flexure and the mass is about to rupture

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Figure 5: Postresection specimen showed giant jejunal gastrointestinal stromal tumor with an adequate bowel margin on either side

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Figure 6: Postoperative histopathology showed spindle-shaped cells with elongated nuclei and eosinophilic cytoplasm

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In the postoperative following with CT and positron emission tomography CT showed no evidence of recurrence. The patient has been followed up for more than 2 years. During follow-up, the patient developed minor complications such as photosensitivity.

Discussion

The symptoms of small intestine GIST vary from pain abdomen, abdominal distension, vomiting, blood vomiting, melena, mass abdomen, lower gastrointestinal bleeding, small bowel obstruction, perforation peritonitis, and symptoms related to acute abdomen.^[2] The small bowel GIST more than 4 cm usually produces symptoms due to perforation, bleeding, and obstruction. The chance of tumor rupture increased when the tumor size is more than 4 cm.^[3] The surgical resection will be considered as R1 if the tumor is allowed to rupture. The tumor rupture or perforation is associated with poor prognosis. Hence, prompt surgical intervention will avoid this complication.

Microscopically, GIST is classified into three types: They are (a) spindle morphology (most common), (b) epithelioid, and (c) mixed of spindle and epithelioid type.^[4] The most characteristic marker of GIST is CD 117 (C-kit) and it is expressed by nearly 100%. But still, there are reports of GIST with negative CD 117 (C-kit) also. These tumors behave aggressively and associated with a high recurrence rate.^[5] The next important marker used in GIST is CD 34 which is expressed around 60%. The recent marker used in GIST is DOG 1 (Discovered on GIST 1). DOG 1 is a monoclonal antibody used to detect GIST. It is expressed around 75%–85% of GIST.^[6]

The small bowel GIST is associated with the poor outcome because of the (1) late presentation, (2) difficult to diagnose, (3) large size of the presentation, (4) aggressive tumor biology, (5) older age of presentation, (6) high lymph node metastasis, (7) poor prognosis, and (8) high recurrence rate. GIST arises from the small intestine usually has a delayed presentation to the health-care providers and it is due to the free mobile small bowel, large mass needed to produce symptoms, and it is easily missed by clinical examination.^[7] The following factors are considered high-risk factors for the patient with GIST. The dependent risk factor includes (1) spindle-epithelioid histology, (2) small bowel GIST, (3) age of the person, (4) genetics of the tumor, (5) immunohistochemistry, (6) morphology of tumor, (7) Type of resection-R0 or R1, (8) tumor spillage, and (9) tumor rupture. The independent risk factors include (1) tumor size more than 5.3 cm and (2) nuclear division >5/50 HPF.

In the diagnostic investigations, contrast CT can detect the small bowel GIST around 90% whereas ultrasound abdomen detects the small bowel GIST around 30%. Gastrointestinal endoscopy is the least useful investigation to diagnose the small bowel GIST. The detection rate of the small bowel GIST is around 20%–25%. The reason behind the low diagnostic yield is poor access to the small bowel by an endoscopy. It may need either push enteroscopy or capsule endoscopy for further evaluation. Hence, the primary investigation modality for the small bowel GIST is contrast CT.

The preferred treatment of the small bowel GIST is surgery. For localized GIST complete surgical resection is the treatment of choice.^[8] The role of lymphadenectomy in the small bowel GIST limited because of the low incidence of lymph node metastasis. Laparoscopic surgery is one of the accepted surgical modalities of treatment for the small bowel GIST <5 cm. GIST larger than 5 cm has a high chance of tumor rupture and spillage inside the abdominal cavity following laparoscopy. Apart from surgery targeted therapy is also an accepted treatment for recurrent disease and metastatic disease. For metastatic and locally advanced GIST, chemotherapy with tyrosine kinase inhibitors like Imatinib is advised. Chemotherapy and radiotherapy are not much useful in GIST.^[9]

Conclusion

In conclusion, we present an unusual case of large jejunal GIST arises from the antimesenteric border. Most of the small intestinal GIST present as a mobile mass in the right iliac fossa. Hence, a patient with mobile right iliac fossa mass without signs of obstruction or minimal signs of obstruction should consider the small bowel GIST as one of the differential diagnosis and plan for prompt intervention. The small bowel GIST can be suspected if the patient has the following triad. (1) abdominal pain, (2) mobile right iliac fossa mass, and (3) no or minimal signs of intestinal obstruction. Good preoperative imaging will diagnose small intestine GIST. Proper preoperative diagnosis and timely intervention will reduce the complication like tumor rupture, spillage, R1 resection, and local recurrence. Postsurgical chemotherapy with Imatinib will reduce the recurrence rate and increase survival.

Learning points/take home messages

Most of the small intestinal GIST present as a mobile mass in the right iliac fossa
A patient with mobile right iliac fossa mass without signs of obstruction or minimal signs of obstruction should consider the small bowel GIST as one of the differential diagnosis and plan for prompt intervention
The small bowel GIST can be suspected if the patient has the following triad: (1) abdominal pain; (2) Mobile right iliac fossa mass; and (3) No or minimal signs of intestinal obstruction
Good preoperative imaging will diagnose small intestine GIST
Proper preoperative diagnosis and timely intervention will reduce the complication such as tumor rupture, spillage, R1 resection, and local recurrence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Lim KT, Tan KY. Current research and treatment for gastrointestinal stromal tumors. World J Gastroenterol 2017;23:4856-66.
2.	Arata R, Nakahara H, Urushihara T, Itamoto T, Nishisaka T. A case of a diverticulum-like giant jejunal gastrointestinal stromal tumour presenting with intraperitoneal peritonitis due to rupture. Int J Surg Case Rep 2020;69:68-71.
3.	Zhao L, Zhao Z, Wang W, Zhao W, Tuo S, Shi Y, et al. Current characteristics on small intestinal stromal tumor - A case control study. Ann Palliat Med 2020;9:98-107.
4.	Dhull AK, Kaushal V, Dhankhar R, Atri R, Singh H, Marwah N. The inside mystery of jejunal gastrointestinal stromal tumor: A rare case report and review of the literature. Case Rep Oncol Med 2011;2011:985242.
5.	Boşoteanu M, Bălţătescu GI, Deacu M, Aşchie M, Boşoteanu CA. A rare case of a double high-risk gastrointestinal stromal tumor of jejunum with KIT-negative/PDGFRA-positive immunophenotype. Rom J Morphol Embryol 2019;60:963-70.
6.	Zhou L, Liao Y, Wu J, Yang J, Zhang H, Wang X, et al. Small bowel gastrointestinal stromal tumor: A retrospective study of 32 cases at a single center and review of the literature. Ther Clin Risk Manag 2018;14:1467-81.
7.	Dualim DM, Loo GH, Rajan R, Nik Mahmood NR. Jejunal GIST: Hunting down an unusual cause of gastrointestinal bleed using double balloon enteroscopy. A case report. Int J Surg Case Rep 2019;60:303-6.
8.	Sashidharan P, Matele A, Matele U, Al Felahi N, Kassem KF. Gastrointestinal stromal tumors: A case report. Oman Med J 2014;29:138-41.
9.	Sankey RE, Maatouk M, Mahmood A, Raja M. Case Report: Jejunal gastrointestinal stromal tumour, a rare tumour, with a challenging diagnosis and a successful treatment. J Surg Case Rep 2015;2015:rjv050.