Urgent surgical treatment of GIST of esophago-gastric junction in a patient with giant hiatal hernia

Abstract

Gastrointestinal stromal tumors (GISTs), being the most common mesenchymal tumors of the gastrointestinal tract, arise most commonly in stomach (60–70%) and small intestine (20–25%) while other sites of origin are rare. In most cases, they are dia gnosed accidentally due to their indolent clinical course; however, 10–30% have malignant potential. Gastric and esophageal GISTs carry a better prognosis than small bowel GISTs of similar size and mitotic rate. Complete surgical resection is the only potentially curative procedure, but despite its success, at least 50% of patients develop recurrence or metastases. Tyrosine kinase inhibitor imatinib gave positive results in treatment of unresectable, metastatic or recurrent GISTs. We present the case of a 69-year-old woman with a large unresectable GIST of esophago-gastric junction with multiple bilobar liver metastases who underwent an emergent palliative surgery due to diff use bleeding from the tumor. Twelve months after the surgery, patient is still alive and stable under imatinib therapy with no signs of local recurrence of the disease. This example suggests that patients with locally advanced GISTs with distant metastases may benefi t from surgery in terms of prolonged survival and quality of life.