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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 6  |  Issue : 1  |  Page : 45-46

Gastrointestinal stromal tumor presenting as perforative peritonitis


Department of Surgery, Mahatma Gandhi Medical College and Hospital, Jaipur, Rajasthan, India

Date of Web Publication15-Feb-2017

Correspondence Address:
Siddharth Pramod Dubhashi
A2/103, Shivranjan Towers, Someshwarwadi, Pashan, Pune - 411 008, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-344X.200204

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  Abstract 

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors arising from the muscularis propria of the gastrointestinal tract. Presenting features include abdominal mass (5%–50%), obstruction (5%), hemorrhage and rarely perforation (0.8%). This is a case report of an elderly male with a perforated jejunal GIST presenting as acute abdomen. GISTs exhibit an unpredictable behavior. Complete surgical excision with clear margins is the treatment of choice with adjuvant chemotherapy.

Keywords: Gastrointestinal stromal tumor, jejunum, perforative peritonitis


How to cite this article:
Dubhashi SP, Jenaw R. Gastrointestinal stromal tumor presenting as perforative peritonitis. Int J Health Allied Sci 2017;6:45-6

How to cite this URL:
Dubhashi SP, Jenaw R. Gastrointestinal stromal tumor presenting as perforative peritonitis. Int J Health Allied Sci [serial online] 2017 [cited 2020 May 26];6:45-6. Available from: http://www.ijhas.in/text.asp?2017/6/1/45/200204

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors arising from the muscularis propria of the gastrointestinal (GI) tract. They are found in the stomach (50%), small intestine (25%), colon (10%), esophagus (5%), and outside GI tract (10%).[1],[2] Histologically, GISTs consist of spindle cell, pleomorphic or epithelioid tissue.[3] GISTs are benign in 70%–80% cases.[4] Presenting features include abdominal mass (5%–50%), obstruction (5%), hemorrhage and rarely perforation (0.8%).[5]


  Case Report Top


A 65-year-old male presented to the emergency department with pain in abdomen, vomiting, constipation, and abdominal distension for 2 days. On examination, there was evidence of pallor, tachycardia (120/min), generalized tenderness over distended abdomen and absent bowel sounds. Per rectal examination was within normal limits. X-ray abdomen revealed gas under both domes of diaphragm. Resuscitation with Ryle's tube, intravenous fluids and antibiotics was done. The patient then underwent emergency laparotomy which revealed peritoneal contamination with purulent fluid. There were purplish colored growths-one in the ileum (around 10 cm proximal to ileocecal junction) and two in the jejunum 1 (one around 60 cm and second around 110 cm from duodenojejunal flexure). There was evidence of central perforation in the distal jejunal growth. Meckel's diverticulum around 15 cm proximal to ileocecal junction was an incidental finding [Figure 1]. Two resections were performed one which included both the jejunal growths and the second one included the ileal growth along with the Meckel's diverticulum. End to end anastomosis was done following both the resections. Peritoneal lavage was given with warm normal saline. Tube drains were placed and the abdomen was closed in layers. The postoperative period was uneventful.
Figure 1: Purplish growths in jejunum and ileum with perforation in jejunal growth

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Histopathological examination revealed poorly differentiated malignant neoplastic lesions, with marked nuclear pleomorphism, dense cellularity and areas of necrosis. There was evidence of infiltration into surrounding adipose tissue. The resected areas were free from tumor invasion. The picture was consistent with a spindle cell GIST [Figure 2]. Immunohistochemical studies revealed positive staining for kit (CD117). The patient was advised treatment with imatinib, which he refused and expired 2 months following surgery.
Figure 2: H and E stained section (×40) composed of relatively uniform spindle cells in short fascicles or whorls. Cytoplasm is pale eosinophilic and nuclei are uniform and ovoid with vesicular chromatin

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  Discussion Top


GIST was first described by Mazor and Clark in 1983.[5] These lesions arise from the interstitial cells of Cajal located in the muscularis propria. There are sporadic as well as familial forms with autosomal dominant inheritance.[6] Ninety percent of GISTs occur above 40 years of age with a slight male predominance.[7] Spontaneous perforation of jejunal GIST with peritonitis is a rare presentation.[5] Perforation occurs due to the replacement of bowel wall by tumor cells, tumor embolization leading to ischemia, necrosis along with raised intraluminal pressure.[7],[8]

Several published reports describe perforated GISTs of high malignant potential.[4] Chatterjee et al.[9] have described three cases of perforated GISTs in elderly males. Histopathologically, the lesions are composed of spindle (70%), epitheloid and round cells or an admixture. Immunohistochemical studies for CD117 antigen are the mainstay for diagnosis.[10]

Surgical treatment includes complete removal of tumor with clear surgical margins, which would entail a 5-year survival of around 60%.[5],[10] Perforation of the tumor carries a grave prognosis due to peritoneal dissemination.[11] There is a definitive role for adjuvant chemotherapy with imatinib, which is a selective tyrosine kinase inhibitor with a dosage of 400 mg/day for 12 months. A long-term follow-up is essential with positron emission tomography with 18F-fluoro-2-deoxy-D-glucose being a useful tool in patients treated with imatinib.[9]


  Conclusion Top


GISTs exhibit an unpredictable behavior. Perforated GIST presenting as peritonitis, though rare, should be included in the differential diagnosis as acute abdomen. Complete surgical excision with clear margins is the treatment of choice with adjuvant chemotherapy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Laurini JA, Carter JE. Gastrointestinal stromal tumors: A review of the literature. Arch Pathol Lab Med 2010;134:134-41.  Back to cited text no. 1
    
2.
DcMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 2000;231:51-58.  Back to cited text no. 2
    
3.
Burkill GJ, Badran M, Al-Muderis O, Meirion Thomas J, Judson IR, Fisher C, et al. Malignant gastrointestinal stromal tumor: Distribution, imaging features, and pattern of metastatic spread. Radiology 2003;226:527-32.  Back to cited text no. 3
    
4.
Skipworth JR, Fanshawe AE, West MJ, Al-Bahrani A. Perforation as a rare presentation of gastric gastrointestinal stromal tumours: A case report and review of the literature. Ann R Coll Surg Engl 2014;96:96E-100E.  Back to cited text no. 4
    
5.
Efremidou EI, Liratzopoulos N, Papageorgiou MS, Romanidis K. Perforated GIST of the small intestine as a rare cause of acute abdomen: Surgical treatment and adjuvant therapy. Case report. J Gastrointestin Liver Dis 2006;15:297-9.  Back to cited text no. 5
    
6.
Miettinen M, Sobin LH, Lasota J. Gastrointestinal stromal tumors presenting as omental masses – A clinicopathologic analysis of 95 cases. Am J Surg Pathol 2009;33:1267-75.  Back to cited text no. 6
    
7.
Sornmayura P. Gastrointestinal stromal tumors (GISTs): A pathology view point. J Med Assoc Thai 2009;92:124-35.  Back to cited text no. 7
    
8.
Karagülle E, Türk E, Yildirim E, Gõktürk HS, Kiyici H, Moray G. Multifocal intestinal stromal tumors with jejunal perforation and intra-abdominal abscess: Report of a case. Turk J Gastroenterol 2008;19:264-7.  Back to cited text no. 8
    
9.
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 Dr NTR Univ Health Sci 2013;2:118-21.  Back to cited text no. 9
    
10.
Roy SD, Khan D, De KK, De U. Spontaneous perforation of jejunal gastrointestinal stroma tumor (GIST). Case report and review of literature. World J Emerg Surg 2012;7:37.  Back to cited text no. 10
    
11.
Steigen SE, Bjerkehagen B, Haugland HK, Nordrum IS, Løberg EM, Isaksen V, et al. Diagnostic and prognostic markers for gastrointestinal stromal tumors in Norway. Mod Pathol 2008;21:46-53.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2]



 

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