Spontaneous perforation of jejunal GIST: Rare presentation of the rare tumor in a rural hospital

Sharvani Kanakraddi, Ajit Kanakraddi. venkatesh Hospital

Introduction: GISTs are rare group of tumors originating from interstitial Cajal cells of digestive tract. They account for 0.1–3% of all gastrointestinal tumors and have a frequency of10- 20/1,000,000 population; they were previously identified as leiomyomas, leiomyosarcomas or leiomyoblastomas; and now are reclassified on immunohistochemical features, with a positive expression of Kit (CD117 antigen). They are usually located in the stomach (60%), small intestine (20-30%) and 10% occur in other parts of the GI tract.  Generally, GISTs have a silent behavior and are diagnosed incidentally; they are difficult to diagnose and often advanced at the time of definitive treatment. Symptoms are highly dependent on the size and location of the tumor. They are characterized by indolent clinical symptoms including vague abdominal pain, weight loss, occult GI bleeding and obstruction, however, we present a case of perforated GIST located in the jejunum as a rare cause of acute abdomen. Spontaneous perforation of GIST is an extremely rare presentation and occurs due to haemorrhagic necrosis.

Typically, GISTs tend to invade locally and spread by direct extension into adjacent tissues and rarely hematogenously to the liver, lungs, and bone; lymphatic metastases are unusual; their malignant potential is 20-30% The most useful indicators of survival and the risk for metastasis include the size of the tumor at presentation, the mitotic index, and evidence of tumor invasion into the lamina propria.

Case Presentation: A 50 year old male presented with sudden onset of pain abdomen, vomiting and fever since 3days.0/E he was haemodynamically stable and had tenderness, guarding and rigidity all over abdomen. Complete haemogram was normal. Ultrasound abdomen revealed only mild ascites; X-ray erect abdomen had air under the right diaphragm. During exploratory laparotomy there was a perforated exophytic growth of size 6x 6 cm which was communicating with lumen of jejunum; the growth was around 15 cm from DJ flexure. After adequate peritoneal lavage, tumuour was resected with 5cm margin and end to end anastomosis of bowel was done. HPR revealed GIST with R0 clearance. Immunohistochemistry was positive for CD-117 antigen. Patient is on oral imatinib since 3 months.

Discussion: There have been only 15 reported cases of perforated GISTS in the literature. Complete removal with postoperative imatinib therapy entails optimal treatment. The 5-year survival rate is 35%. It increases to 54% after complete surgical excision. However 40% will recur within 18 – 24 months. Once recurrence has occurred median survival is 9–16 months.

 

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