A huge esophageal Schwannoma occurring in a Caucasian young male: a case report

Gastrointestinal schwannomas are rare benign mesenchymal tumors originating from Schwann cells, the nerve sheath belonging to the Auerbach's plexus or, less frequently, to Meisser's plexus. The esophagus is the least common site accounting for less than 2% of all esophageal tumors, and the upper to mid portion is usually involved. Esophageal schwannomas affect more frequently middle-aged Asian women. The most common symptom is dysphagia. Diagnosis requires histological and immunohistochemical studies and the standard of care is surgical resection.

We present the case of a 22-year-old Caucasian male who was admitted to our hospital for progressive dysphagia and acute chest pain. An EGDS showed an elongated bulging of the lower esophagus with signs of a subcentimetric mucosal erosion. A CT-scan showed a lower esophageal ectasia and a huge postero-lateral wall mass measuring 37x28x70 mm. An endoscopic ultrasonography showed a hypoechoic heterogeneous mass with multiple anechoic areas and a fine needle biopsy was performed. Histological examination showed tissue made up of spindle cells with mild eosinophilic cytoplasm and rare nuclear atypia, which were intensively and diffusely positive for the S100 protein on immunohistochemical studies thus allowing pre-operative diagnosis of "ancient" schwannoma. after a multidisciplinary discussion, the patient underwent a surgical resection. Since the tumor had a transmural extension, a subtotal esophagectomy was performed to achieve complete resection with negative margins.

This is the first case of a young Caucasian male patient with an "ancient" schwannoma of the lower esophagus, a benign but locally advanced lesion treated by subtotal esophagectomy.