Riffle Mary Ellen, Polydorides Alexandros D, Niakan Jessica, Chehade Mirna
Department of *Pediatrics, Mount Sinai Center for Eosinophilic Disorders, Jaffe Food Allergy Institute †Pathology, Icahn School of Medicine at Mount Sinai, New York, NY.
Am J Surg Pathol. 2017 May;41(5):616-621. doi: 10.1097/PAS.0000000000000832.
Esophageal granular cell tumors (GCTs) are rare. Eosinophilic esophagitis (EoE) is an immune-mediated disease characterized by esophageal eosinophilia despite proton pump inhibitor (PPI) therapy. Given that GCTs occur at sites of scarring and inflammation, we sought to determine the prevalence of EoE in patients with esophageal GCTs. Our center's pathology database was searched for GCT specimens from 1995 to 2014. Slides were blindly rereviewed. GCTs were scored for atypical cytological features. Presence and number of eosinophils in the tumor and the surrounding esophageal epithelium and any EoE features were recorded. Medical records were reviewed. From >30,000 esophageal cases, 23 esophageal GCTs were identified, with 18 available for review (16 adult, 2 pediatric). Median patient age was 38.7 years. Four adults had esophageal intraepithelial eosinophilia (peak 38 to 68 eosinophils/high power field [HPF]); 2 confirmed to have EoE, 1 with PPI-responsive esophageal eosinophilia, and 1 had not received PPI therapy. Both pediatric cases had confirmed EoE (peak 24 and 34 eosinophils/HPF). In total, 12/18 GCTs had intratumoral eosinophilia (peak 1 to 16 eosinophils/HPF). All 6 cases with esophageal eosinophilia had intratumoral eosinophilia. Two GCTs displayed atypical cytologic features. Esophageal eosinophilia was present in 25% of adult and 100% of pediatric GCTs, the majority confirmed to have EoE. Overall, 67% of cases had intratumoral eosinophilia and 2 had atypical features. On the basis of these findings, we propose evaluating surrounding tissue for eosinophilia when esophageal GCT is diagnosed, and adding GCT as a potential complication of untreated EoE. Research for an immunologic link between EoE and esophageal GCTs is needed.
食管颗粒细胞瘤（GCTs）较为罕见。嗜酸性粒细胞性食管炎（EoE）是一种免疫介导性疾病，其特征为即便接受质子泵抑制剂（PPI）治疗，食管仍存在嗜酸性粒细胞浸润。鉴于GCTs发生于瘢痕形成和炎症部位，我们试图确定食管GCT患者中EoE的患病率。检索了本中心1995年至2014年的病理学数据库中的GCT标本。对玻片进行盲法复查。对GCTs的非典型细胞学特征进行评分。记录肿瘤及周围食管上皮中嗜酸性粒细胞的存在情况和数量以及任何EoE特征。查阅了病历。在超过30000例食管病例中，识别出23例食管GCTs，其中18例可供复查（16例成人，2例儿童）。患者中位年龄为38.7岁。4例成人存在食管上皮内嗜酸性粒细胞增多（峰值为每高倍视野38至68个嗜酸性粒细胞）；2例确诊为EoE，1例为PPI反应性食管嗜酸性粒细胞增多，1例未接受PPI治疗。2例儿童病例均确诊为EoE（峰值分别为每高倍视野24个和34个嗜酸性粒细胞）。总共12/18例GCTs存在瘤内嗜酸性粒细胞增多（峰值为每高倍视野1至16个嗜酸性粒细胞）。所有6例存在食管嗜酸性粒细胞增多的病例均有瘤内嗜酸性粒细胞增多。2例GCTs表现出非典型细胞学特征。25%的成人GCTs和100%的儿童GCTs存在食管嗜酸性粒细胞增多，大多数确诊为EoE。总体而言，67%的病例有瘤内嗜酸性粒细胞增多，2例有非典型特征。基于这些发现，我们建议在诊断食管GCT时评估周围组织是否存在嗜酸性粒细胞增多，并将GCT作为未经治疗的EoE的一种潜在并发症。需要对EoE与食管GCTs之间的免疫联系进行研究。
