Background:Pediatric granular cell tumors (GCT) involving the gastrointestinal tract (GIT) are rare with limited case report/series reported to date.Methods:Multicenter retrospective study of pediatric GIT GCT.Results:A total of 10 cases were included in the study with a median age of 13.5 years (range: 7-18 years) and were predominantly female patients (60%). In half of the patients no significant medical history was present with the remaining 5 having Crohn disease (10%), eosinophilic esophagitis (EoE) (10%), Crohn disease and EoE (10%), growth hormone deficiency (10%), and aplasia cutis congenita (10%). The GCT median size was 1.3 cm (range: 1-1.6 cm) and were more commonly located in the esophagus (70%) followed by the stomach (20%) and rectum (10%). Most of the cases showed round/polygonal tumor cells with abundant granular cytoplasm, and none of the cases had nuclear atypia, increased mitotic activity, or tumor cell necrosis. None of our cases received specific therapy for GCT other than clinical follow-up, and none of the patients had evidence of local recurrence or metastatic disease.Conclusion:We present our multicenter experience with GIT GCT, all cases had a benign course. Interestingly, 4 of the esophageal GCT cases (including 2 patients with EoE) showed an eosinophil-rich esophagitis in the underlying mucosa.

中文翻译：

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儿童胃肠道颗粒细胞肿瘤：多中心经验

背景：累及胃肠道（GIT）的小儿颗粒细胞瘤（GCT）很少见，迄今为止的病例报告/系列报道有限。方法：小儿 GIT GCT 的多中心回顾性研究。结果：共纳入 10 例研究中位年龄为 13.5 岁（范围：7-18 岁），主要为女性患者（60%）。一半患者没有明显的病史，其余 5 名患者患有克罗恩病 (10%)、嗜酸性粒细胞性食管炎 (EoE) (10%)、克罗恩病和 EoE (10%)、生长激素缺乏症 (10%)、和先天性皮肤发育不全（10%）。GCT 中位大小为 1.3 厘米（范围：1-1.6 厘米），更常见于食道（70%），其次是胃（20%）和直肠（10%）。多数病例肿瘤细胞呈圆形/多角形，细胞质丰富，颗粒状，无一例出现核异型性、有丝分裂活性增强或肿瘤细胞坏死。除临床随访外，我们的病例均未接受针对 GCT 的特异性治疗，并且没有患者有局部复发或转移性疾病的证据。结论：我们介绍了 GIT GCT 的多中心经验，所有病例均为良性病程。有趣的是，4例食管GCT病例（包括2例EoE患者）显示底层粘膜存在富含嗜酸性粒细胞的食管炎。