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ESOPHAGEAL GASTROINTESTINAL STROMAL TUMORS: A SINGLE-INSTITUTION EXPERIENCE
Arianna Barbetta*1, Tiffany J. Garcia1, Micheal J. Cavnar2, Manjit S. Bains1, Bernard J. Park1, Ronald P. DeMatteo2, David R. Jones1, Daniela Molena1
1Department of Thoracic Surgery, Memorial Sloan Kettering Cancer Center, New York, NY; 2Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY
Introduction: Although gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the digestive tract, they are still rare compared with all the gastrointestinal malignancies. The esophageal location is particularly uncommon, occurring only in less than 1% of cases, leading to very limited data on treatment and prognosis. The aim of this study was to evaluate outcome of patients with esophageal GIST and identify clinicopathologic features with a prognostic value.
Methods: Patients treated for esophageal GIST from 1973 to 2016 were identified from an institutional prospective database. All patients with a histologically confirmed tumor were included, regardless of treatment plan. Patient’s demographics, tumor characteristics, treatments and survival outcomes were analyzed. Data are expressed as median (Interquartile Range).
Results: From 1973 to 2016, 25 esophageal GIST were treated at our institution. The median age of patients at diagnosis was 62 years (55-70). Eighteen patients (72%) underwent resection: 5 (28%) had tumor enucleation and 13 (72%) had esophagectomy. Five patients (20%) were treated with definitive Imatinib and 2 patients had no treatment. The median greater diameter of resected tumors was 7.2cm (5-10), whereas it was 4.9cm (1.75-6.15) for the non-surgical patients (p=0.05). Among patients who underwent resection, 7(39%) received Imatinib either as neoadjuvant therapy (2 patients), adjuvant (4 patients) or both (1 patient). The mitotic index was greater than 5/50 HPF in 47% of cases and the median mitotic count per 50HPF was 5 (2-15). Molecular studies performed on 19 patients showed the most common site of mutation to be exon 11 (37%). The median disease-free survival among surgical patients was 3.6 years (0.7-6.12).
Conclusion: Due to paucity of data, the prognosis of esophageal GIST is not well established. Consequently, the best therapeutic approach is unknown. Our series shows that esophageal GIST is perhaps a more aggressive malignancy than commonly perceived and therefore a multidisciplinary approach to evaluate and treat these patients is warranted.
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