Pediatric Neuroendocrine Neoplasms: Rare Malignancies with Incredible Variability.
Pediatric Neuroendocrine Neoplasms: Rare Malignancies with Incredible Variability.
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神经内分泌肿瘤的发病率正在以惊人的速度增加，这意味着更多的供应商在成人和儿科患者中遇到它们。这种肿瘤分类包括各种不同的恶性肿瘤，表现为各种症状，每种都有不同的治疗方法。此外，在过去几年中，这些肿瘤的分类发生了变化，治疗方法也发生了各种变化和进展。鉴于这一点及其在儿科患者中的罕见性，医疗保健提供者可能不熟悉这些变化。我们这次审查的目的是提供儿科患者中所有最常见的神经内分泌肿瘤形式的概述，并提供最新的建议，以便任何医疗保健提供者能够快速准确地适应自己。神经内分泌肿瘤（NEN）包括各种神经内分泌肿瘤（NET）和神经内分泌癌（NEC），它们可以发生在身体的任何地方。虽然在儿科人群中相对罕见，但NEN的发病率在过去几十年中有所增加。这些肿瘤如果没有早期诊断和治疗，可能是毁灭性的，然而，症状是可变的，可以是多年的惰性。据报道，从出现第一个症状到诊断的时间中位数为10年。考虑到其中一些肿瘤的死亡率高达90%，医疗保健提供者必须意识到NEN并保持警惕。通过更好的提供者教育和容易获得的关于这些肿瘤的信息资源，可以提高认识，从而更早地识别和诊断疾病。本文旨在概述儿科人群中最常见的NEN以及罕见的高致死性NEN。该综述提供了最新的证据和建议，包括最近的分类变化和治疗方式的进展，包括最近完成和正在进行的临床试验。
Neuroendocrine neoplasms are increasing in incidence at a remarkable rate meaning more providers are encountering them in both adult and pediatric patients. This classification of neoplasm encompasses a wide range of different malignancies with a variety of symptoms at presentation and each treated differently. Additionally, over the past few years there has been a change in classification of these neoplasms and a variety of changes and advances in how they are treated. Given this and their rarity in pediatric patients, healthcare providers may not be familiar with these changes. Our goal with this review was to provide an overview of all the most commonly encountered forms of neuroendocrine neoplasms in pediatric patients with up to date recommendations so any healthcare provider can quickly and accurately acclimate themselves. Neuroendocrine neoplasms (NENs) encompass a variety of neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) which can arise anywhere in the body. While relatively rare in the pediatric population, the incidence of NENs has increased in the past few decades. These neoplasms can be devastating if not diagnosed and treated early, however, symptoms are variable and can be indolent for many years. There is a reported median of 10 years from the appearance of the first symptoms to time of diagnosis. Considering some of these neoplasms have a mortality rate as high as 90%, it is crucial healthcare providers are aware of NENs and remain vigilant. With better provider education and easily accessible resources for information about these neoplasms, awareness can be improved leading to earlier disease recognition and diagnosis. This manuscript aims to provide an overview of both the most common NENs as well as the rarer NENs with high lethality in the pediatric population. This review provides up to date evidence and recommendations, encompassing recent changes in classification and advances in treatment modalities, including recently completed and ongoing clinical trials.
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影响因子：
0.9
作者：
Cogen, Jonathan D.;Swanson, Jonathan;Ong, Thida
通讯作者：
Ong, Thida
影响因子：
28.4
作者：
通讯作者：
--
影响因子：
3.3
作者：
Bellizzi, Andrew M.
通讯作者：
Bellizzi, Andrew M.
影响因子：
3.6
作者：
Cholewa, D;Waldschmidt, J;Wiedenmann, B
通讯作者：
Wiedenmann, B
DOI：
10.1159/000512089
发表时间：
2021-10-01
期刊：
GE-Portuguese Journal of Gastroenterology
影响因子：
--
作者：
Carvão, Joana;Dinis-Ribeiro, Mário;Libânio, Diogo
通讯作者：
Libânio, Diogo