关键词: esophagus gastrointestinal tract granular cell tumor pediatric rectum stomach

Mesh : Humans Granular Cell Tumor / pathology diagnosis Adolescent Female Child Male Retrospective Studies Gastrointestinal Neoplasms / pathology diagnosis

来  源:   DOI:10.1177/10935266231220472

Abstract:
UNASSIGNED: Pediatric granular cell tumors (GCT) involving the gastrointestinal tract (GIT) are rare with limited case report/series reported to date.
UNASSIGNED: Multicenter retrospective study of pediatric GIT GCT.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要:
涉及胃肠道(GIT)的小儿颗粒细胞瘤(GCT)很少见,迄今报道的病例报告/系列报道有限。
小儿GITGCT的多中心回顾性研究。
共有10例病例纳入研究,中位年龄为13.5岁(范围:7-18岁),主要为女性患者(60%)。在一半的患者中,没有明显的病史,其余5人患有克罗恩病(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(10%),和先天性表皮发育不全(10%)。GCT的中位尺寸为1.3cm(范围:1-1.6cm),更常见于食道(70%),其次是胃(20%)和直肠(10%)。大多数病例表现为圆形/多边形肿瘤细胞,颗粒细胞质丰富,这些病例都没有核非典型,有丝分裂活性增加,或者肿瘤细胞坏死.除临床随访外,我们的病例均未接受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。
我们展示了GITGCT的多中心体验,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)显示下层粘膜中富含嗜酸性粒细胞的食管炎。
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