A Review for the Clinician: Classifications, Genetics, and Treatment for Neuroendocrine Neoplasms of the Thymus (Thymic Carcinoids).

IF 3.2 2区医学 Q2 ENDOCRINOLOGY & METABOLISM

Neuroendocrinology Pub Date : 2025-03-11 DOI:10.1159/000544982

Matthias Lang, Chrysanthi Anamaterou, Isabelle Mohr, Mária Černá, Manuel Röhrich, Christine Tjaden

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引用次数: 0

Abstract

Background: Thymic carcinoids or neuroendocrine neoplasms (t-NEN) are a rare entity with a dismal prognosis. About 25% of the tumors are related to multiple endocrine neoplasia type I (MEN-1), where they contribute significantly to mortality. The tumors are classified according to the WHO classification, TNM classification and Masaoka-Koga staging system, although none of the classifications have been developed for t-NEN. A recently proposed t-NEN specific morphomolecular classification is based on copy number instability scores. Its role is yet to be defined. The prognosis depends on resectability, histological features, metastasis, the amount of copy number instabilities and mitotic activity.

Summary: No study-based therapies exist. The mainstay of therapy is surgical resection as it is associated with significantly improved long-term survival. Based on published cases and small series, for non-resectable and recurring disease, platinum-based chemotherapies are preferred in neuroendocrine carcinoma, while everolimus and temozolomide are recommended in thymic neuroendocrine tumors.

Key messages: This review covers current classification systems and the knowledge of genetic disorders and medical therapies.

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临床综述：胸腺神经内分泌肿瘤（胸腺类癌）的分类、遗传学和治疗。

这篇综述涵盖了目前的分类系统和遗传疾病的知识和医学治疗。胸腺类癌或神经内分泌肿瘤（t-NEN）是一种罕见的预后不佳的肿瘤。约25%的肿瘤与I型多发性内分泌瘤（MEN-1）有关，它们对死亡率有显著影响。肿瘤根据WHO分类、TNM分类和Masaoka-Koga分期系统进行分类，尽管目前还没有针对t-NEN的分类。最近提出的t-NEN特异性形态分子分类是基于拷贝数不稳定性评分。它的作用还有待界定。预后取决于可切除性、组织学特征、转移、拷贝数不稳定性和有丝分裂活性。目前还没有基于研究的治疗方法。治疗的主要是手术切除，因为它与显著改善的长期生存有关。根据已发表的病例和小系列研究，对于不可切除和复发性疾病，神经内分泌癌（t-NEC）首选铂类化疗，而胸腺神经内分泌肿瘤（t-NET）推荐依维莫司和替莫唑胺。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neuroendocrinology 医学-内分泌学与代谢

CiteScore

8.30

自引率

2.40%

发文量

审稿时长

6-12 weeks

期刊介绍： ''Neuroendocrinology'' publishes papers reporting original research in basic and clinical neuroendocrinology. The journal explores the complex interactions between neuronal networks and endocrine glands (in some instances also immunecells) in both central and peripheral nervous systems. Original contributions cover all aspects of the field, from molecular and cellular neuroendocrinology, physiology, pharmacology, and the neuroanatomy of neuroendocrine systems to neuroendocrine correlates of behaviour, clinical neuroendocrinology and neuroendocrine cancers. Readers also benefit from reviews by noted experts, which highlight especially active areas of current research, and special focus editions of topical interest.