儿童的初级肝神经内分泌瘤,一个系统的审查
Ali Samady Khanghah1, Omid Madadi-Sanjani2, Anahita Abdolzadeh1
1Department of Surgery, School of Medicine, Ardabil University of Medical Sciences, Ardabil, Iran.
Journal of neuroendocrinology
|February 10, 2025
概括
主要肝神经内分泌瘤 (PHNEN) 是一种罕见的儿科瘤. 手术切除为这些罕见的肝脏神经内分泌瘤提供了最成功的长期结果.
科学领域:
- 肝胆细胞新生体的发生.
- 儿科瘤学 儿科瘤学
- 神经内分泌瘤 神经内分泌瘤
背景情况:
- 神经内分泌新生体 (NENs),以前称为癌类瘤,包括多种瘤组.
- 主要肝神经内分泌瘤 (PHNENs) 异常罕见,仅占所有NENs的0.4%.
- 区分PHNEN与转移性神经内分泌瘤到肝脏至关重要,因为二次参与更为常见.
研究的目的:
- 审查和分析儿科原发性肝神经内分泌瘤 (PHNENs) 记录的病例.
- 了解儿童PHNEN的临床表现,诊断方法和治疗结果.
- 突出儿童群体中PHNENs的罕见性和特征.
主要方法:
- 在主要的科学数据库 (PubMed/Medline,科学网,Scopus,谷歌学者) 进行全面的文献搜索.
- 包括关于儿科PHNEN (年龄在19岁以下的患者) 的全文英语文章.
- 排除尸检结果,癌症和孤立的胆囊干扰.
主要成果:
- 总共有10例儿科PHNEN病例被确定.
- 患者的平均年龄为13.86岁 (范围为8-19岁),女性轻微占主导地位 (55%).
- 右叶是最常见的部位,腹痛是主要症状,CT扫描有助于诊断.
结论:
- 儿科PHNEN是罕见但独特的实体,需要仔细诊断.
- 系统性评估是强制性的,以排除转移性疾病在诊断一次性肝脏瘤为NEN之前.
- 手术切除是实现长期成功的最有效的治疗策略,在儿科PHNENs.
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