|Year : 2013 | Volume
| Issue : 2 | Page : 118-121
A rare presentation of gastrointestinal stromal tumors as small bowel perforation: A single institution based clinical experience of three cases
Chandan Chatterjee, Dawood Khan, Utpal De
Department of General Surgery, Medical College and Hospital, Kolkata, India
|Date of Web Publication||21-May-2013|
46 Dr. Jagabandhu Lane, Kolkata – 700012, West Bengal
Source of Support: None, Conflict of Interest: None
Gastrointestinal stromal tumors (GIST) of intestine presenting as acute abdomen due to perforation of the tumor is extremely rare.We present three cases of jejunal GIST which presented as acute abdomen resulting from perforation of the tumor. All the three cases underwent emergency laparotomy and resection of the tumor with clear margin. Histopathological examination was compatible with GIST and c-Kit/CD 117 was positive in immunohistochemistry. All the three patients received adjuvant chemotherapy with imatinib.
Keywords: Acute abdomen, gastrointestinal stromal tumors, jejunal perforation
|How to cite this article:|
Chatterjee C, Khan D, De U. A rare presentation of gastrointestinal stromal tumors as small bowel perforation: A single institution based clinical experience of three cases. J NTR Univ Health Sci 2013;2:118-21
|How to cite this URL:|
Chatterjee C, Khan D, De U. A rare presentation of gastrointestinal stromal tumors as small bowel perforation: A single institution based clinical experience of three cases. J NTR Univ Health Sci [serial online] 2013 [cited 2020 Mar 31];2:118-21. Available from: http://www.jdrntruhs.org/text.asp?2013/2/2/118/112339
| Introduction|| |
Gastrointestinal stromal tumors (GISTs) are rare but they are the most common mesenchymal tumors of the gastrointestinal tract. It is estimated that the frequency of these tumors is 10-20 per 1 million population.  The possibility of presence of malignancy is 20-30%. Primary GIST can arise throughout the gastrointestinal tract. However, the most common location of GIST is stomach (50-60%) and small intestine(30-40%). 
Many GISTs are asymptomatic, but patients with advanced disease may present with a mass lesion or vague abdominal pain or obstruction. Their first clinical manifestation as acute abdomen due to perforation is extremely rare. In emergency, laparotomy a diagnosis of GIST should be kept in mind and R0 resection with adjuvant chemotherapy should be considered.
| Case Reports|| |
A 50-year-old male patient presented with diffuse abdominal pain for 8 hours, vomiting, and abdominal distension. His body surface temperature was 38°C.There was no history of hematemesis or melena. Hematologic examination revealed elevated leukocytes count. Straight X-ray of chest showed free gas under diaphragm. After proper resuscitation of the patient, emergency laparotomy was planned. On exploration, there was diffuse peritonitis with a 6 × 7 cm exophytic growth found in the antimesenteric border of jejunum 10 cm distal to the ligament of Treitz with a small perforation near the base of the tumor. The tumor was firm in consistency with irregular surface and patchy areas of hemorrhage and necrosis in it. The tumor was resected out taking clear margin on both sides and primary end-to-end anastomosis was done. Histopathology report was consistent with GIST having more than five mitotic figure/50 high power field (HPF) and C-kit/CD117 positive in immunohistochemistry (IHC). The patient received adjuvant chemotherapy with imatinib.
A 55-year-old male was presented in the casualty with pain in the upper abdomen and distension for the last 2 days.There was no history of vomiting, fever. Free gas was found under the diaphragm on straight X-ray of chest and leukocytosis was detected on hematological examination. Exploratory laparotomy was done after proper fluid electrolyte correction of the patient.On exploration, it was found that there was bilious peritoneal collection.Thorough examination of the gut revealed a 4 × 5 cm exophytic growth with firm consistency and irregular surface,which was present in the antimesenteric border of the jejunum approximately 25 cm distal from ligament of Treitz. There was a perforation near the base of the growth. Resection of the tumor was done with clear margin and primary anastomosis was done. Histopathological examination was in favor of GIST with more than five mitotic figure/50 HPF and C-kit/CD117 positivity in IHC. Adjuvant chemotherapy with imatinib was advocated for the patient.
A 62-year-old male patient presented in the emergency department with diffuse abdominal pain and distension for 2 days. There was fever for the last 1 day. There was no history of hematemesis, vomiting and melena. Free gas was detected under the diaphragm in straight X-ray of chest.Elevated leukocyte count was observed in complete hemogram. Exploratory laparotomy was done.On exploration, huge bilious peritoneal collection was found. There was a 10cm × 7 cm exophytic growth with irregular surface and firm consistency, present in the antimesenteric border of jejunum 30 cm away from ligament of Treitz. There was a small perforation near the base of the tumor.Resection of the tumor was done with clear margin and primary anastomosis was done.Histopathology report was suggestive of GIST with more than five mitotic figure/50 HPF. IHC was positive for CD117/C-kit.The patient received adjuvant chemotherapy with imatinib.
| Discussion|| |
In the present study, we have reported three cases of jejunal GIST presenting as acute abdomen due to perforation of the tumor.
GISTs are the most common sarcomatous tumors of gastrointestinal tract. They are known to be a distinct tumor arising from interstitial cells of Cajal, an intestinal pacemaker cell. Approximately, 70% of GISTs arise in the stomach, with 20-30% arising from small intestine and remaining 10% from esophagus, colon, rectum, omentum, mesenteries, and retroperitoneum. , Velasco et al., reported that the jejunal GIST accounts for approximately 10% of all the cases.  The clinical presentation of GIST is erratic. Further more, only 70% of patients are symptomatic, where as 20% are asymptomatic and 10% are detected at autopsy.  In the review of Miettinen and Lasota, the most common presentation is reported as gastrointestinal bleeding.  Spontaneous perforation of jejunal GIST is unique and rare. A similar kind of case has been reported recently by Fenget al., 2011.  The tumors smaller than 2 cm in size are generally asymptomatic and larger tumors maybe present with upper abdominal pain, palpable intra-abdominal mass, vomiting, weight loss, perforation, or rupture. Rupture of GISTs into peritoneal cavity is rare and it causes life-threatening intraperitoneal hemorrhage.  Karagülle et al., suggested that the reason of the perforation or rupture of GIST is the replacement of bowel walls by tumor cells followed by necrosis, ischemia of the intestine due to tumor embolization, and increased intraluminal pressure caused by obstruction.  In our report, all the three patients presented with features of peritonitis.On exploration, all the three patients were found to have an exophytic growth in the antimesenteric border of jejunum with irregular surface, firm consistency, and areas of hemorrhage and necrosis within it. In all cases there was a perforation at the base of the tumor [Figure 1].
|Figure 1: Exophytic growth in the antimesenteric border of jejunum with a perforation at its base|
Click here to view
Although there are various investigation modalities for diagnosing GIST none of them can establish the correct diagnosis with 100% certainty. Histopathology with IHC can confirm the diagnosis. IHC examination of GIST is always positive for c-Kit (CD117 antigen)[Figure 2], while the positivity regarding other markers (CD34, SMA, S100, Desmin) varies.  GIST can be categorized as low-risk or high-risk depending on the possibility of metastasis and recurrence. However, the main prognostic factor is mitotic count and size.In our report, all three cases had tumor size >5 cm and >5 mitotic figure/50 HPF. According to prognostic classification defined by Fletcher et al., all the three cases in our study fall in the high-risk category. 
|Figure 2: Immunohistochemistry of the growth showing positivity for CD117|
Click here to view
Surgical resection of local disease is the gold standard therapy. Its goal is complete resection of the disease with avoidance of tumor rupture and spillage.  Complete surgical resection allows 48-65% possibility of 5-year survival.  Along with surgical resection there is definite role of adjuvant chemotherapy with imatinibmesylate (Glivec® FDA approval May 2001), a selective tyrosine kinase inhibitor. According to a prospective study conducted by American College of Surgeons Oncology Group (ACOSOG) tolerable dose of imatinib was 400 mg/day for 12 months. 
GISTs have an unpredictable behavior, and a long-term follow-up is essential for all patients, independent of their benign or malignant characteristic. According to the National Comprehensive Cancer Network guideline,contrast CT of the abdomen and pelvis is recommended every 3-6 months for 3-5 years and then yearly.Positron emission tomography is a very useful tool for postoperative follow-up of patients receiving imatinib.
| Conclusion|| |
Though rare, GIST in the jejunum may present as acute abdomen resulting from perforation of tumor. So possibilities of GIST should be kept in mind in male patients of fifth to sixth decade presenting with acute abdomen due to perforation of a jejunal mass near its base in the antimesenteric border with firm consistency, irregular surface, and areas of hemorrhage and necrosis in it. Oncologic surgery principles including clear resection margins and resection without spillage or rupture should be tried in such cases. Based on histopathology and IHC adjuvant chemotherapy with imatinib and close follow-up should be advocated.
| Acknowledgement|| |
Dr. Utpal Kr. Dutta, Principal, Medical College and Hospital.
| References|| |
|1.||Joensuu H, Kindblom LG. Gastrointestinal tumours-A review.Acta Orthop Scand Suppl 2004;75:62-71. |
|2.||Stamatakos M, Douzinas E, Stefanaki C, Safioleas P, Polyzou E, Levidou G, et al. Gastrointestinal stromal tumours. World JSurg Oncol 2009;7:61. |
|3.||Sunamak O, Karabicak I, Aydemir I, Aydogan F, Guler E, Cetinkaya S, et al. An intraluminal leiomyoma of small intestine causing invagination and obstruction: A case report. Mt Sinai J Med 2006;73:1079-81. |
|4.||Bucher P, Egger JF, Gervaz P, Ris F, Weintraub D, Villiger P, et al. An audit of surgical management of gastrointestinal stromal tumours(GIST). Eur J Surg Oncol 2006;32:310-4. |
|5.||Velasco S, Millin S, Maurel C, Richer JP, Sylvain C, Hannequin J, et al. Scanographic features of gastrointestinal stromal tumours.Gastroenterol Clin Biol 2008;32:1001-13. |
|6.||Kim KM, Kang DW, Moon WS, Park JB, Park CK, Sohn JH, et al. Gastrointestinal Stromal Tumour Committee; Korean Gastrointestinal Pathology Study Group. Gastrointestinal stromal tumours in Koreans: Its incidence and the clinical, pathological and immunohisto chemical findings. J Korean Med Sci 2005;20:977-84. |
|7.||Miettinen M, Lasota J. Gastrointestinal stromal tumours: Pathology and prognosis at different sites. Semin Diagn Pathol 2006;23:70-83. |
|8.||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. |
|9.||Motegi A, Sakurai S, Nakayama H, Sano T, Oyama T, Nakajima T. PKC theta, a novel immunohistohemical marker for stromal tumours (GIST), especially useful for identifying KIT-negative tumours.PatholInt 2005;55:106-12. |
|10.||Karagülle E, Türk E, Yildirim E, Gõktürk HS, Kiyici H, Moray G. Multifocal intestinal stromal tumours with jejuna perforation and intra abdominal abscess: Reportof a case. Turk J Gastroenterol 2008;19:264-7. |
|11.||Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. J Clin Oncol 2004;22:3813-25. |
|12.||Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, et al. Diagosis of Gastrointestinal stromal tumours: A consensus approach. Hum Pathol 2002;33:459-65. |
|13.||Verweij J, Casali PG, Zalcberg J, LeCesne A, Reichardt P, Blay JY, et al. Progression free survival in gastrointestinal stromal tumours with high-dose imatinib: Randomised trial. Lancet 2004;364:1127-34. |
|14.||Parfitt JR, Streutker CJ, Riddell RH, Driman DK. Gastrointestinal stromal tumours. A comtemporary review. Pathol Res Pract 2006;202:837-47. |
|15.||Li FP, Fletcher JA, Heinrich MC, Garber JE, Sallan SE, Curiel-Lewandrowski C, et al. Familial gastrointestinal stromal tumours syndrome: Phenotypic and molecular feature in a kindred. J Clin Oncol 2005;23:2735-43. |
[Figure 1], [Figure 2]