Malignant granular cell tumor of the cervical esophagus with loco-regional lymph node metastases

Granular cell tumors (GCTs) are uncommon, mostly benign lesions. The malignant form and involvement of the cervical esophagus are rare. A 53-year-old female was referred to our hospital for treatment of a malignant cervical esophageal GCT. We performed a pharyngolaryngeal esophagectomy, total thyroidectomy and selective bilateral neck lymph node dissection. Histopathology revealed a malignant GCT involving cervical esophagus. S100, CD56, inhibin and PGP9.5 proteins were positive and the surgical margins were negative. Metastatic disease in 6 loco-regional lymph nodes and focal infiltration in tracheal tissue were detected. To the best of our knowledge, this is the first case of malignant GCT arising in cervical esophagus showing loco-regional lymph node metastases. At present the patient is free of disease 7 months after surgery.