Gastrointestinal stromal tumor presenting as perforative peritonitis

Siddharth Pramod Dubhashi; Ratnesh Jenaw

doi:10.4103/2278-344X.200204

CASE REPORT

Year : 2017 | Volume : 6 | Issue : 1 | Page : 45-46

Gastrointestinal stromal tumor presenting as perforative peritonitis

Siddharth Pramod Dubhashi, Ratnesh Jenaw
Department of Surgery, Mahatma Gandhi Medical College and Hospital, Jaipur, Rajasthan, India

Date of Web Publication

15-Feb-2017

Correspondence Address:
Siddharth Pramod Dubhashi
A2/103, Shivranjan Towers, Someshwarwadi, Pashan, Pune - 411 008, Maharashtra
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2278-344X.200204

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 2023 Nov 29];6:45-6. Available from: https://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

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

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

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.

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