Esophageal gastrointestinal autonomic nerve tumor.

Abstract

Esophageal gastrointestinal stromal tumors (GISTs) are less common than other tumors of the esophagus. Gastrointestinal autonomic nerve tumors (GANTs) comprise a subgroup of GISTs. These tumors are mostly located in the small intestine and stomach, whereas they rarely arise in the esophagus. In immunohistochemical examinations, staining for CD117 and CD34 is positive in these tumors, whereas smooth muscle actin staining is negative. The most important risk factors for progressive disease are the mitotic count and tumor size, and surgery is recommended for the treatment of suitable cases. In this article, we discussed the clinicopathologic features of GISTs and the treatment options in light of the literature of a patients diagnosed with esophageal GANTs. Gastrointestinal stromal tumors (GISTs) have been reported to be anatomically located in the stomach; small intestine; colon, rectum, and appendix; and esophagus in 51-70%, 25-36%, 5-7%, and 1-3% of cases, respectively (1). Gastrointestinal autonomic nerve tumors (GANTs) comprise a subgroup of GISTs. These tumors are mostly located in the small intestine and stomach, whereas they rarely arise in the esophagus. On computed tomography of the thorax of a 61-yearold female patient who presented to our clinic with shortness of breath and swallowing difficulty, a lesion of approximately 62 × 33 × 68 in size was observed. The lesion filled the prevertebral distance and subcarinal area from the level of the arcus aorta in the mediastinum and compressed the right and left main bronchi, and it had borders with the esophagus that could not be distinguished. A diagnosis could not be made, and as a result, transbronchial biopsy was performed. The maximum standardized uptake value on positron emission tomography-computed tomography (PET/CT) of the mass was 23.8 (Figure 1a). There was no pathologic involvement for the mass on PET/CT, and the patient underwent right thoracotomy. The lesion, which appeared to originate from between the muscle fibers of the esophagus, was excised without complete removal of the esophageal mucosa (Figure 1b). On immunohistochemical examination of the tumor, in which microscopically spindle cells were generally organized as cellular areas, CD117 and CD34 staining was positive, and in addition to the existence of neuronal cells and S-100 positivity, staining for smooth muscle actin, desmin, and synaptophysin was negative (Figure 2). The mass was 7 cm in size with 1 mitotic count per 50 high-power fields (HPFs). The tumor was histopathologically diagnosed as a GANT, and the risk for progressive disease as intermediate.