Background Granular cell tumor of the esophagus is a rare submucosal neoplasm. We present here four patients with granular cell tumors (GCTs) of the esophagus who were seen and treated at our institution.
Methods Retrospective review of endoscopic and pathology databases was undertaken to identify GCTs of the esophagus. Medical records were reviewed for clinical and surgical treatment. A median follow-up of 31 months (range 8-43 months) was available.
Results Four male patients with a median age of 53 years (range 50-58 years) were identified with a diagnosis of GCTs of the esophagus between February 2001 and February 2004. This diagnosis of GCTs constituted only 0.001% of all patients who underwent an upper endoscopy during that interval. All patients were asymptomatic for dysphagia. Indication of upper endoscopy was reflux symptoms (1), iron deficiency anemia (1), and abnormal CT scan findings of esophageal lesion with celiac lymphadenopathy (2). On upper endoscopy GCTs appear as small submucosal nodule or polypoid lesion in the esophagus with size varying from 5-15 mm. Endoscopic biopsies were positive for histologic diagnosis of GCTs in three out of four patients. Three patients underwent surgical resection due to size of the tumor and/or associated lymphadenopathy. Surgical specimen confirmed the diagnosis of GCTs in all patients. Lymph nodes were negative for any metastatic disease in all 3 cases. One patient with a small 5 mm lesion (positive endoscopic biopsy) was followed clinically. During follow-up, in the surgical group one patient died 8 months postoperatively unrelated to GCT, one patient developed atrial-esophageal fistula 23 months postoperatively and required surgery with cervical esophagostomy, and one remained clinically stable at 27 months postoperatively. One patient followed clinically remained asymptomatic on his last clinic follow-up at 43 month.
Conclusions Esophageal granular cell tumors are infrequent lesions identified on upper endoscopy. They are mostly benign, although malignant degeneration has been reported. Endoscopically they appear as submucosal lesions but a histologic diagnosis can be obtained by endoscopic biopsies in majority of cases. Asymptomatic, smaller lesions require observation only but larger or symptomatic lesions usually require surgical resection. Although regional lymphadenopathy can be seen on radiologic studies with GCTs, lymph node histologic assessment failed to show any tumor involvement.
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