We describe the successful surgical management of a rare case of esophageal GIST rupture. We believe this case is notable for several points. Firstly, the case is educational because the patient’s presenting symptoms closely mimicked that of esophageal perforation, a condition characterized by chest or back pain accompanied by mediastinal enlargement and pleural effusion on imaging studies. Notable deviations from the usual presentation of perforation were the lack of free air findings on imaging and the relatively mild pain experienced by the patient. In such cases, esophageal tumor rupture should be considered as a differential diagnosis.

Furthermore, an esophageal GIST rupturing into the thoracic cavity instead of the esophageal lumen is a very rare phenomenon. GISTs rupturing into the gastrointestinal lumen are not uncommon. For example, gastrointestinal bleeding due to tumor invasion is a relatively common presentation of esophageal GIST, with a reported occurrence rate of one in eight cases [12]. In stark contrast, only one case of hemothorax caused by an esophageal GIST rupturing into the thoracic cavity has ever been described in literature [9]. Interestingly, in our patient and in the previously reported case, the tumor was situated the caudal mediastinum below the tracheal bifurcation and grew in an extramural fashion. We postulate that this location allowed the tumor to grow to significant size without compressing the esophagus or surrounding structures, thus progressing unnoticed, until finally rupturing into the thoracic cavity.

Another noteworthy aspect of this case is that we conducted an emergent operation. Esophageal GISTs do not typically present as surgical emergencies, and hence, there are very few published examples of management in such situations [4,5,6,7,8]. One such example by Romic et al. reported an emergent “en bloc” resection of a 11 × 10 cm esophagogastric junction GIST due to uncontrollable GI bleeding after initiation of palliative imatinib therapy [7]. In our case, the profuse hemorrhage and massive size of the tumor significantly limited our options, which led to a “piecemeal”, and potentially incomplete, resection. However, the piecemeal resection was successful in controlling hemorrhage, rescuing the patient’s life, and returning him to normal daily life.

Finally, the case begs the discussion of whether incomplete resection has survival benefit in esophageal GIST. Because of the rarity of esophageal GIST, the studies to guide us on this topic do not exist. One paper that may inform us is a retrospective study of 239 patients with metastatic GIST who were on imatinib therapy and who were also provided with incomplete resection or debulking surgery of the tumor [13]. Surgery did not prolong survival in this study. As the tumor in our patient was not metastatic, we cannot confidently comment on the benefit of incomplete resection for our case. However, it is not implausible that in non-metastatic cases such as ours, even incomplete resection could have benefit by debulking the tumor. Since we had to discontinue imatinib, the prognosis of this patient is further complicated. There is evidence from the pre-imatinib era that 80% of GIST relapse occurred within 24 months of surgery [14]. Because the patient has shown no sign of disease progression at 10 months, we remain cautiously optimistic. Regardless of the lack of prognostic evidence for esophageal GIST, we believe our case is a valuable data point to guide clinicians in the future.

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