关键词: Eosinophilic esophagitis Esophagus Granular cell tumor Pediatric S100 SOX10

Mesh : Humans Male Child Female Immunohistochemistry Granular Cell Tumor / complications pathology Esophageal Neoplasms / complications pathology Biopsy

来  源:   DOI:10.1093/ajcp/aqad025

Abstract:
Granular cell tumor (GCT) commonly presents in the subcutaneous tissue and head and neck region, and it is uncommon in the gastrointestinal tract. Experience with esophageal GCTs in the pediatric population is limited, with only 7 cases reported in the literature, 3 with eosinophilic esophagitis (EoE).
Case information from 11 pediatric patients with GCTs of the esophagus was retrieved. H&E and immunohistochemical slides were reviewed with clinical, endoscopic, and follow-up data from all patients.
In total, 7 male and 4 female patients were included, with ages ranging from 3 to 14 years. Indications for esophagogastroduodenoscopy (EGD) included EoE (n = 3), follow-up for Crohn disease, and other nonspecific complaints. Endoscopically, all patients had a single submucosal, firm mass protruding into the lumen, with normal overlying mucosa. The nodules were removed endoscopically in multiple fragments in all cases. Histologically, the tumors showed sheets and trabeculae of cells containing bland nuclei, inconspicuous nucleoli, and abundant pink granular cytoplasm without atypical features. All tumors were immunoreactive for S100, CD68, and SOX10. Follow-up showed that all patients were disease-free (median, 2 years).
We report the largest series of pediatric esophageal GCTs with coincidental association with EoE. These EGD findings are characteristic, and removal by biopsy is both diagnostic and therapeutic.
摘要:
目的:颗粒细胞瘤(GCT)通常出现在皮下组织和头颈部,在胃肠道中并不常见。在儿科人群中使用食道GCT的经验有限,文献中只有7例报道,3伴嗜酸性粒细胞性食管炎(EoE)。
方法:检索了11例食管GCT患儿的病例资料。H&E和免疫组织化学切片进行了临床回顾,内窥镜,和所有患者的随访数据。
结果:总计,包括7名男性和4名女性患者,年龄从3到14岁不等。食管胃十二指肠镜检查(EGD)的适应症包括EoE(n=3),克罗恩病的随访,和其他非特定投诉。内窥镜检查,所有患者都有单一的粘膜下,伸入管腔的坚固物质,粘膜正常。在所有情况下,都通过内窥镜将多个碎片中的结节切除。组织学上,肿瘤显示细胞的片状和小梁含有温和的细胞核,不显眼的核仁,和丰富的粉红色颗粒细胞质,无非典型特征。所有肿瘤对S100、CD68和SOX10均具有免疫反应性。随访显示所有患者均无病(中位数,2年)。
结论:我们报告了与EoE有巧合关联的最大系列小儿食管GCT。这些EGD发现是有特点的,活检切除是诊断和治疗。
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