Abstract
BackgroundEsophageal granular cell tumor (eGCT) is rare, and the recent literature suggests a link between eosinophilic esophagitis (EoE) and eGCT. The aim of our study was to determine if EoE or other disorders associated with eosinophilia are consistently associated with eGCT.MethodsWe retrospectively searched pathology databases of three academic institutions from 1999 to 2018 for eGCTs. The archived slides and medical records were reviewed.ResultsFrom 294,855 esophagogastroduodenoscopy procedures, 45 patients (17 males and 28 females) with eGCTs were identified. The patients (30–73 years in age, median 50) had eGCT 0.2–2.0 cm in size (average 0.71). Thirteen had a history of gastroesophageal reflux disease, 5 had Barrett esophagus/goblet cell metaplasia and 1 had EoE. Thirty-four eGCTs had intralesional eosinophils (14 with peak > 10 eosinophils/400x hpf); of these, 21 also had eosinophils in lamina propria (9 with peak > 10 eosinophils/hpf). eGCT with atypical features (including nuclear enlargement and prominent nucleoli) were more likely to have increased eosinophils in non-epithelial compartments than those without atypia. Pleomorphism and spindled cells were seen in 3 eGCT cases (mean peak intralesional eosinophils: 43 per hpf); 2 of these had goblet cell metaplasia. We found no association between EoE and eGCT, p = 0.5966, (95% C.I. 0.0276, 6.5389, Fisher’s exact test). Instead, most patients had gastroesophageal reflux disease or Barrett esophagus.ConclusionEosinophilia, common in eGCT and adjacent stroma, likely drives atypical/reactive histologic features, but a pathogenic relationship between eosinophil rich inflammatory conditions and eGCT has not yet been established.
Highlights
Esophageal granular cell tumor is rare, and the recent literature suggests a link between eosinophilic esophagitis (EoE) and eGCT
Considering all EGD procedures performed at the three institutions with a diagnosis of EoE and eGCT, we found no association between EoE and eGCT, [p =
Recent studies have reported the co-occurence of eGCT and significant esophageal intraepithelial eosinophilia, most frequently in the pediatric population [9, 11, 12]
Summary
Esophageal granular cell tumor (eGCT) is rare, and the recent literature suggests a link between eosinophilic esophagitis (EoE) and eGCT. Granular cell tumor (GCT) was first described in the tongue by Abrikossoff in 1926. Other names for this entity are granular cell myoblastoma and Abrikossoff’s tumor [1, 2]. Eosinophilic esophagitis (EoE) is a clinicopathologic disease that has been more consistently recognized, with increasing prevalence, since the initial publication of consensus recommendations in 2007 [6]. EoE is a chronic, immune/antigen-mediated disease with eosinophil-predominant inflammation that leads to esophageal dysfunction [7]. GCTs have been previously linked to sites of injury and inflammation, leading some to postulate that these lesions might be reactive in nature [8]. There have been several reported cases of concomitant esophageal granular cell tumor (eGCT) and
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