A Statistically Significant Association Between Esophageal Granular Cell Tumors and Eosinophilic Esophagitis: A 16-year Analysis at Two Large Hospitals of 167,434 EGDs.

BACKGROUND: Esophageal granular cell tumor (eGCT) is a rare, usually benign, neoplasm of neuroectodermic origin. Eosinophilic esophagitis (EoE) is a relatively uncommon, immune-mediated, chronic disease. Both diseases commonly present with dysphagia. One case has been reported of simultaneous occurrence of both diseases.

AIMS: To determine the association between diseases.

METHODS: The present study was an IRB-approved, retrospective review of esophagogastroduodenoscopies (EGDs) with esophageal biopsies from two large hospitals, 1999-2014.

RESULTS: Among 29,235 EGDs with esophageal biopsies for 16 years (167,434 total EGDs), 16 patients had pathologically diagnosed eGCT, and 1225 patients had pathologically diagnosed EoE. Five (31%) of 16 patients with eGCT had concomitant EoE (p = 0.001, OR 10.43, 95% ORCI 3.16-32.44, Fisher's exact test). Patients with simultaneous eGCT and EoE were young (mean age = 33.6 ± 12.9 years). Three were female. Dysphagia was presenting symptom in 4 (80%) of patients. Three had asthma. All five patients had > 20 eosinophils/hpf in esophageal biopsy specimens. Three patients had endoscopic esophageal abnormalities suggesting EoE. Four patients were treated with a PPI (before and after diagnosis of EoE), and 2 patients underwent six-food-elimination diet with partial symptomatic improvement. The eGCTs averaged 13.4 ± 4.2 mm in maximal diameter and were located in upper-2, middle-2, and lower esophagus-2 (1 patient had 2 eGCTs). eGCTs were endoscopically resected-3 patients, and monitored-2 patients. Surveillance endoscopies revealed no recurrence or growth of eGCTs after resection (mean follow-up = 4.6 years).

CONCLUSIONS: This novel report of 5 patients with simultaneous EoE and eGCT adds to one, previously published case and suggests these two diseases are associated, and have a common pathophysiologic link, despite apparently different pathogenesis. Large, prospective, endoscopic and pathologic studies are warranted to further investigate this association.