Large esophageal schwannoma: En-bloc resection with primary closure by esophagoplasty()

BACKGROUND: Gastrointestinal schwannomas are submucosal tumors accounting for 2–7% of mesenchymal gastro-intestinal neoplasms; the stomach being the most common site. Esophageal schwannomas are more frequent in women, and are usually located in the upper to mid portion. Dysphagia is the main presenting symptom. A definitive diagnosis requires confirmation by histopathological and immunohistochemical studies. CASE PRESENTATION: A 50-year-old healthy lady, presented with gradual increasing onset of dyspnea, with minimal dysphagia to solid food, over a period of several years. Enhanced CT scan of the chest revealed a well-defined soft tissue mass arising from the proximal third of the esophagus, measuring 7.8 × 5.4 x 10.5 cm. Esophagogastric endoscopy with ultrasonography showed an elevated, smooth surface lesion, arising from the submucosal layer of the esophagus, with a hypervascular mucosa. Enucleation of this large tumor, with preservation of the overlying mucosa, was difficult to accomplish due to its large size. Making use of a dilated proximal esophageal segment, total en-bloc excision of the mass rendered a 15 cm esophagotomy gap, which was easily closed, in two layers, without affecting the overall caliber thus achieving a good esophagoplasty result. Histologically, abundance of spindle-shaped cells with positive S-100 proteins, confirmed the diagnosis of esophageal schwannoma. CONCLUSION: Variations in mesenchymal gastrointestinal tumors is vast, rendering diagnosis by radiology alone difficult. As such, characteristic histologic and immunostaining features are cornerstones in precise diagnosis of esophageal schwannomas. Despite being rare in incidence, symptomatic esophageal schwannoma lesions can be excised entirely, with low rate of recurrence and favorable overall outcomes.