Granular Cell Tumors of the Esophagus: A Clinical and Pathologic Study of 13 Cases

Abstract

Fewer than 200 cases of esophageal granular cell tumors (GCT) have been reported. However, determination of malignant potential and appropriate management are as yet unresolved issues. We evaluated the clinical and pathologic features of 13 esophageal GCTs. This group included 11 women and 2 men. Six patients were black. Patient ages ranged from 27 to 69 years (median, 47 years). Tumors were found incidentally in 10 patients. Only 2 patients were symptomatic. Most tumors were in the distal esophagus and were less than 8 mm. In 5 patients, multiple GCTs were found, including 3 patients with multiple esophageal GCTs. In 11 cases, the tumor was diagnosed by endoscopic biopsy. Ten patients were treated with biopsy alone, without further attempt at tumor excision. No recurrent or metastatic disease developed within the follow-up period. In all but 1 case, tumors were characterized by nests of cells with pyknotic nuclei, abundant granular cytoplasm, an absence of mitotic figures, and strong S-100 protein positivity. One patient who presented with dysphagia had a 2.5-cm distal esophageal GCT that showed atypical histologic features, including cellular spindling, nuclear pleomorphism, and mitoses. Because of this patient's symptoms, the large tumor size, and atypical endoscopic ultrasonographic and histologic features, the lesion was surgically excised. Esophageal GCTs are usually asymptomatic, small, and found incidentally on upper endoscopy done for other reasons. Flexible esophagoscopy and biopsy are the mainstay in diagnosis, and endoscopic ultrasound can provide additional information on the layer of origin and tumor extension. Observation of these tumors is indicated unless the patient is symptomatic or the tumor is greater than 1 cm or has atypical endoscopic ultrasonographic or histologic features.