Granular cell tumor of the cervical esophagus: case report and literature review of an unusual cause of Dysphagia

Abstract

Granular cell tumors (GCT) of the head and neck are not uncommon; however, involvement of the cervical esophagus is rare. Characterized by an infiltrative growth pattern, these benign tumors are historically difficult to surgically excise and are radioresistant. We present here a case of dysphagia caused by a GCT of the cervical esophagus. Work up with ultrasound-guided fine needle aspiration was suggestive of a GCT due to the presence of cohesive cells with granular cytoplasm that were S-100 and CD68 positive with immunostaining, and PAS positive with histochemistry. Resection required removal of a portion of the muscular wall of the esophagus sparing the overlying mucosa. The patient is currently asymptomatic and without recurrence after 10 month follow-up. Review of the literature revealed 19 reports of cervical esophageal GCTs. There is a female preponderance (75%), with an average age of 41 years. Dysphagia and weight loss are the most common presenting symptoms. The average tumor size on presentation was 2.7 cm, with symptomatic tumors being significantly larger than asymptomatic lesions; the latter was present in 25% of patients. Concurrent GCTs in the upper aerodigestive tract were identified in 35% of cases. Approximately 30% of tumors required segmental cervical esophageal resection. The purpose of this report is to describe the epidemiology and treatment of GCTs of the cervical esophagus. Lesions should be addressed early with complete surgical excision to prevent growth necessitating more morbid surgery. Due to the high rate of concurrent GCTs, upper endoscopy is advised in the workup of these patients.

Fig. 1

a Immunohistochemical staining for S-100 protein on cell block derived from needle rinse of fine needle aspiration (40x). b Section of granular cell tumor from resection specimen showing closely packed large cells with indistinct borders and granular cytoplasm and bland nuclei. (H and E stain, 20x)

Fig. 2

a, b Antero-posterior (a) and lateral (b) views from pre-operative esophagram showing extrinsic compression of barium column postero-laterally on the left side (arrows). c, d Antero-posterior (c) and lateral (d) views from post-operative esophagram, showing resolution of extrinsic compression

Fig. 3

a Pre-operative CT scan revealing homogeneous soft tissue mass in cervical esophagus with compromise of esophageal lumen by GCT (arrow). b Post-operative CT scan revealing patent esophagus