Esophageal GIST: therapeutic implications of an uncommon presentation of a rare tumor.

Gastrointestinal stromal tumors (GISTs) are rarely reported in the esophagus. The authors report a patient with an esophageal GIST, incidentally found after an echocardiogram. CT scan and endoscopic ultrasonography showed the tumor in the dependence of the muscularis propria of the esophageal wall. An Ivor-Lewis esophagectomy was performed. The tumor was well-circumscribed involving the submucosal and the muscular layers of the esophagus, measuring 13.5 x 8.5 x 7.6 cm, without involving the surgical margins. Histologically, the tumor consisted of spindle cells, with low mitotic index (2/50 HPF), that were immunoreactive for KIT (CD117) and CD34, consistent with GIST of high risk of aggressive behavior. No adjuvant therapy was given to the patient, who is alive and without evidence of disease 1 year after surgery. Since esophageal GISTs are rarely reported in the literature and usually have a poor prognosis, the diagnostic differentiation of these tumors from other more common mesenchymal neoplasms is essential, both for therapeutic and prognostic reasons.