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.

Large Mid-Esophageal Granular Cell Tumor: Benign Versus Malignant

“… 9 Tumors of 2 cm or less are usually asymptomatic and are found incidentally during UGI endoscopy or imaging studies. 10 Tumors more than 2 cm in size are usually symptomatic, the commonest symptom being dysphagia. Less commonly patients present with gastro esophageal reflux disease, dyspepsia, chest pain, cough, nausea, and hoarseness.…”

Gastrointestinal and biliary granular cell tumor: diagnosis and management

“…GCTs can induce pseudoepitheliomatous hyperplasia of overlying squamous epithelium, which can lead to a misdiagnosis of squamous cell carcinoma if the biopsy is superficial [ 24 ]. In one study [ 5 ], 9% of GCTs show pseudoepitheliomatous hyperplasia on biopsy, while another study reported that approximately 50% of GCTs in the esophagus and perianal region showed varying degrees of pseudoepitheliomatous hyperplasia [ 6 ].…”

Esophageal granular cell tumor: evaluation of pathological and clinical findings in series of 7 cases

“…Endoskopi ve EUS ile GCT tanısının kesin olarak verilemeyeceği, histopatoloji ve immünhistokimyasal incelemenin zorunlu olduğu bildirilmektedir. Kimi yayınlarda EUS eşliğinde ince iğne aspirasyonu ile GCT tanısı verildiği gösterilmiş-tir (7) . Olgularımızın ikisinde EUS uygulanmış (Olgu 2 ve Olgu 6) ve aynı işlem seansında lezyonlar rezeke edilmiştir.…”

“…Olguların çoğunun distal yerleşimli olduğu ileri sü-rülmüş, proksimal yerleşimli lezyonların çok seyrek görüldüğü, tüm özofageal lezyonların ancak %4-5'ini oluşturduğu belirtilmektedir (1,7) . Serimizde olguların dördü orta kesim, üçü distal uç yerleşimliydi.…”