benign primary intramural tumors of the esophagus are rare and account for approximately 2% of all esophageal tumors . most of them are esophageal leiomyomas ; less frequent are schwannomas or malignant masses such as gastrointestinal stromal tumors ( gist ) . endoscopic ultrasound biopsy or frozen section biopsy during the operation does not differentiate the tumors . we report a case of benign esophageal schwannoma causing progressive dysphagia in a 37-year - old woman . a 37-year - old woman presented due to symptoms of chest pain during swallowing of solid food for 3 months . the physical examination was noncontributory . computed tomography of the chest showed a soft tissue mass in the lumen of the upper esophagus ( fig . endoscopic ultrasound identified the mass at 19 cm from the incisors , but the biopsy was nondiagnostic . computed tomography scan showing concentric soft tissue lesion surrounding the esophagus bronchoscopy and pulmonary function test of the patient were normal . the right chest was opened through a right anterolateral thoracotomy at the fifth intercostal space . the intramural tumor 3.5  3  3 cm was enucleated without erosion of the mucosa ( fig . the esophageal wall allowed primary closure of the muscular layers , and the native esophagus was preserved . enucleated esophageal schwannoma ( 3.5  3  3 cm ) histopathological examination of the tumor revealed spindle - shaped cells in a fasciculated and disarrayed architecture and nuclei in a palisading pattern ( fig . immunohistochemically the tumor was positive for s100p and gfap , and negative for cd34 , cd117 , sma , desmin and h - caldesmon ( fig . spindle - shaped cells in a fasciculated and disarrayed architecture and nuclei in a palisading pattern . a ) 40 , b ) 200 immunohistochemical studies revealed s100 protein positivity postoperative recovery was uneventful , and there has been no evidence of recurrence to date . esophageal schwannoma is uncommon and difficult to diagnose in preoperative investigations due to its similarity to other intramural esophageal tumors ( leiomyoma , gist , oesophageal cysts or cancer ) . these tumors more frequently develop in women and are often located in the upper and mid esophagus . malignant schwannoma has also been reported [ 1 , 2 ] . symptoms of this tumor include dysphagia , dyspnea [ 1 , 3 ] , chest pain , and hematemesis , and appear when the tumor increases in size . imaging studies ( positron emission tomography , computed tomography , and magnetic resonance imaging ) are useful for the confirmation of esophageal tumor [ 1 , 4 , 5 ] . endoscopic ultrasonography - guided fine needle aspiration biopsy is not always useful for diagnosis , as in our cases . the value of frozen section is limited , and it can lead to diagnostic pitfalls . final diagnosis is possible upon positive immunohistochemical studies for s-100 protein and negative staining for smooth muscle markers such as sma , cd34 , and cd117 . surgical excision of the tumor is the method of choice , because chemotherapy and radiotherapy are ineffective . the option of enucleation is recommended when : the tumor is well encapsulated , a clear margin is achievable , and there is sufficient redundant mucosa to close the defect . in our case enucleation with video - assisted thoracoscopic surgery ( vats ) is possible for small tumors (  2 cm ) . for large tumors (  8 cm ) with broad adjacent to the muscular layer and an extensive mucosal defect , esophagectomy with gastric pull - up is recommended [ 3 , 7 ] .