Case Report A case of gastrointestinal secondary to a small bowel gastrointestinal stromal tumor before causing hemoperitoneum

Gastrointestinal stromal tumors (GISTs) is a rare stromal neoplasm that represent the most common mesenchymal tumor of the GI tract, accounting for 0.2% of all GI malignancies and 5% of all sarcomas.[1,2] GIST originating from interstitial cells of Cajal, which are regulators of gut peristalsis, are preferentially located in the [3] Abstract stomach and the . It is characterized by indolent clinical symptoms including vague abdominal pain, weight loss, GI bleeding and obstruction, which is caused by the growing tumor. The usual presentation of GIST is a GI bleed. Acute presentation of GIST with features of hemoperitoneum has never been reported. We report an unusual case of small bowel GIST in which the patient presented with GI bleed before presenting as hemoperitoneum. Key words: Exophytic, gastrointestinal stromal tumor, hemoperitoneum, small bowel

INTRODUCTION

Gastrointestinal stromal tumors (GISTs) is a rare stromal neoplasm that represent the most common mesenchymal tumor of the GI tract, accounting for 0.2% of all GI malignancies and 5% of all sarcomas.[1,2] About 70% occur in the stomach, 20% in the small intestine and less than 10% in the Praveen S. Padasali, . They rarely occur in other abdominal organs. GISTs are thought to arise from interstitial Edigara Govindaraj1, cells of Cajal,[3] that are normally part of the autonomic nervous system of the intestine. They serve Kasa Somasekhar2 a pacemaker function in controlling motility.

Department of Surgery, GISTs is characterized by indolent clinical symptoms including vague abdominal pain, weight loss, GI Kannur Medical College Hospital, Kannur, Kerala, 1Departments of bleeding and obstruction, which is caused by the growing tumor. The usual presentation of GIST is General Surgery and 2Surgery, a GI bleed. Acute presentation of GIST with features of hemoperitoneum has never been reported. Vijayanagara Institute of Medical We report an unusual case of small bowel GIST in which the patient presented with GI bleed before Sciences, Bellary, Karnataka, India presenting as hemoperitoneum. Address for the Correspondence: Dr. Praveen S. Padasali, CASE REPORT Maitri Galli, Jamkhandi, D‑Bagalkot ‑ 587 301, Karnataka, India. A 45‑year‑old man presented to our emergency department with a week‑long history of dull aching E‑mail: [email protected] lower‑abdominal pain and no other symptoms. The severity of pain had increased over the last 2 days and was accompanied by progressive abdominal distension and vomiting. On clinical examination, Access this article online the patient was pale and diaphoretic and had tachycardia with systolic pressure 90 mmHg.

Website: www.oghr.org Abdominal examination showed abdominal distension, diffuse abdominal tenderness, guarding, and rigidity. Routine laboratory investigation revealed Hb‑6 gm%. Abdominal X‑ray (erect) not showed DOI: 10.4103/2348-3113.139652 gas under right dome of diaphragm. Ultrasound examination showed a 5 × 6 cm mass originating Quick response code: from small bowel with free fluid in the abdomen noted. USG‑guided free fluid aspiration shows blood after repeated aspiration. The patient was hemodynamically stabilized and conservatively managed, and later planned for contrast‑computed tomography of the abdomen for detailed evaluation.

Apart from the above he was a healthy male presented with and malena since 6 months on and off without any co‑morbidity and no history of previous abdominal surgery. Initial investigations included an abdominal ultrasound scan (USG), an esophago‑gastro‑duodenoscopy (OGD) and flexible

Oncology, and Reports| Jan-Jun 2015 | Vol 4 | Issue 1 PB 49 Oncology, Gastroenterology and Hepatology Reports| Jan-Jun 2015 | Vol 4 | Issue 1 Padasali, et al.: A case of gastrointestinal bleeding secondary to a small bowel gastrointestinal stromal tumor before causing hemoperitoneum sigmoidoscopy and colonoscopy were also unremarkable. His malena and hematochezia thought due to healed gastric or duodenal lesion and he was advised to take proton pump inhibitors on a regular basis, which resulted in partial and temporary relief of his symptoms. Although his condition did not impact his lifestyle significantly, his symptoms never subsided completely and almost 2 months following initial presentation repeat OGD and colonoscopy not revealed anything. All of these did not reveal any abnormality and thus planned for diagnostic laproscopy, but the patient was hesitant and refused for surgery. Four months later the patient presented with features of hemoperitoneum.

Finally, 4 months from presentation, the patient was admitted to hospital with hemoperitoneum and underwent a within 48 hours of admission after hemodynamic stabilization with adequate fluid and electrolyte replacement. Blood transfusion Figure 1: Small intestinal exophytic GIST with bleeding was done to make case fit for surgery. A clinical diagnosis of hemoperitoneum probably due to gastroduodenal lesion or rupture of intraabdominal abscess was made, and emergency laparotomy was planned.

On exploration of the abdomen, hemoperitoneum noted of around 500 ml and 5 × 6 cm exophytic mass [Figure 1] originating from the proximal about 30 cm distal to the duodenojejunal flexure. It was a firm, lobulated mass with areas of softening and cystic degeneration with overlying distended veins and small intratumoral [Figure 2]. The tumor was hypervascular, with evidence of recent hemorrhage into the tumor. No evidence of visceral metastasis was found. En bloc excision of the tumor with jejunojejunal anastomosis was performed. Postoperative recovery was uneventful.

Histopathological analysis revealed a densely crowded Figure 2: Small intestinal GIST resected specimen spindle and polygonal cell tumor showing moderate nuclear pleomorphism [Figures 3]. 3‑4 mitoses/HPF were found and the tumor spread into the mucosa and outwards into the serosa. There was no lymph nodal spread. Immunohistochemistry demonstrated positive staining for CD117 (cKit) and negative staining for CD34, S‑100 and chromogranin. Based on the size (5 × 6 cm) and mitosis (3‑4/HPF), the tumor was classified as a gastrointestinal stromal tumor (GIST) of intermediate malignant potential. A CT scan of the chest, abdomen and pelvis performed subsequently did not reveal the presence of metastatic disease. The patient was discharged on the 10th postoperative day and decided not to proceed with any adjuvant treatment after departmental meeting. The patient made an uneventful recovery and was remarkably well after 1 year of follow up; all his previous abdominal symptoms were completely resolved. Figure 3: Small bowel GIST microscopy DISCUSSION constitutes a distinct group of rare GI tract tumors that originate GISTs are rare neoplasms of the GI tract ranking a distant third from the interstitial cells of Cajal.[4] The latter are regulators of gut after adenocarcinomas and lymphomas. GISTs can occur anywhere peristalsis and normally express CD117, which is a product of the in the GI tract. Although morphologically similar to other benign c‑kit proto‑oncogene that encodes a tyrosine kinase receptor, which and malignant smooth muscle and neural stromal tumors, GIST regulates cellular proliferation in GIST.

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GISTs arise from the muscularis mucosa or muscularis propria layers areas that have previously been poorly visualized. Video capsule and most exhibit an endophytic growth pattern, growing within the endoscopy have a role in the evaluation of patients presenting with bowel lumen. The overlying mucosa is usually preserved but larger GI bleeding and should be considered as a diagnostic option in the and more aggressive tumors tend to ulcerate through this. They are assessment of such patients, especially in those patients in whom no submucosal lesions that often grow intraluminally. Sometimes they source can be clearly identified. Clinicians should consider it to use have an extraluminal exophytic component. The stomach (60%) as a complementary diagnostic option to OGD and colonoscopy in is the most common site followed by small intestine (30%), the evaluation of small intestinal GI bleeding although a diagnostic (5%), colon/ (5%) and esophagus (<1%). Primary laparoscopy and CT scan may have helped detect the tumor when mesenteric, omental and retroperitoneal GISTs have also been very small which was initially missed by USG. It could have been reported, but they are quite rare.[5] considered at some stage during the months preceding his final presentation as hemoperitoneum. GIST is observed predominantly in adults at a median age of 58 years. The incidence is equal in men and women. It rarely REFERENCES presents in younger age, but in our case, the patient presented at the 1. Logroño R, Jones DV, Faruqi S, Bhutani MS. Recent advances in cell age of 45 years. Clinical presentation of patients varies according biology, diagnosis, and therapy of gastrointestinal stromal tumor (GIST). to site, size and aggressiveness of the tumor. Symptoms manifest Cancer Biol Ther 2004;3:251‑8. when the tumor is large (>5 cm) or situated in a critical anatomical 2. Miettinen M, Sobin LH, Sarlomo‑Rikala M. Immunohistochemical spectrum of GISTs at different sites and their differential diagnosis with a location (e.g. causing gastric outlet obstruction). Symptoms may reference to CD117 (KIT). Mod Pathol 2000;13:1134‑42. include abdominal pain, mass, , vomiting, anorexia and 3. DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. weight loss. Patients may present with GI bleeding (due to pressure Two hundred gastro‑intestinal stromal tumors: Recurrence patterns and prognostic factors for survival. Ann Surg 2000;231:51‑8. necrosis and ulceration of the overlying mucosa) or with obstructive 4. Kindblom LG, Remotti HE, Aldenorg F, Meis‑Kindblom JM. Gastrointestinal symptoms. An extensive review of the literature regarding GISTs pacemaker cell tumor (GIPACT): Gastrointestinal stromal tumors show not revealed any tumor presenting as hemoperitoneum. Two GISTs phenotypic characteristic of the interstitial cells of Cajal. Am J Pathol 1988;152:1259‑69. out of which one was a gastric perforation and the second one was 5. Bertagnolli MM. Gastrointestinal stromal tumors. In: Zinner MJ, [6,7] jejunal but no tumor presented as hemoperitoneum. So, ours Ashley SW, editors. Maingots Abdominal Operation. 11th ed. Vol. 15. is the only reported case presenting as a jejunal tumor presenting New York: McGraw Hill; 2007. p. 441. 6. Ozgun YM, Ergul E, Sisman IC, Kusdemir A. Gastric adenocarcinoma as exophytic growth with hemoperitoneum. Definitive surgery and GIST (collision tumors) of the stomach presenting with perforation; remains the mainstay of treatment for patients with localized first report. Bratisl Lek Listy 2009;110:504‑5. primary GIST. 7. Feng F, Chen F, Chen Y, Liu J. A rare perforated gastrointestinal stromal tumor in the jejunum: A case report. Turk J Gastroenterol 2011;22:208‑12.

CONCLUSION How to cite this article: Padasali PS, Govindaraj E, Somasekhar K. A case of gastrointestinal bleeding secondary to a small bowel Due to the relative inaccessibility of the small bowel, diagnosis gastrointestinal stromal tumor before causing hemoperitoneum. of small intestinal GI bleeding remains a challenge. Advances in Onc Gas Hep Rep 2015;4:49-51. Nil, None declared. imaging techniques of the small bowel allow us to better evaluate Source of Support: Conflict of Interest:

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