副肿瘤综合征:发病理论、临床特点及治疗方法。

Gaelle Flore Ketchandja Ngonga, Daniela Ferrari, Lorenzo Lorusso, Chiara Gasparetto, Eva Neznama, Manuela D'Abramo, Giovanni Ricevuti
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摘要

副肿瘤综合征是一种少见的疾病,具有不同的发病机制和临床表现,与肿瘤有关,但不是由于肿瘤、转移或其他远处影响所致。本文的目的是在文献的基础上描述主要的副肿瘤综合征(神经学,内分泌代谢学,风湿病学,骨关节学,皮肤学,血液学,血管学和肾病学),相关的发病理论(共同胚胎速写理论,信息再激活理论和自身免疫理论)和最重要的治疗方法。实验工作,评论和临床观察,在某些情况下仍在进行中,对所描述的综合征,其发病机制和治疗方法进行了审查。文献中尚未发表关于副肿瘤综合征的荟萃分析。较好描述的发病机制是自身免疫性的,以神经、肾脏和一些皮肤综合征为特征,临床和实验室结果得到了很好的支持。与其他证候相关的发病理论在文献的基础上进行了关联。副肿瘤综合征很重要,因为它们的识别允许肿瘤的早期诊断和快速治疗,大大改善了患者的预后和预期寿命。它们通常是无声肿瘤的唯一信号;有时它们先于肿瘤本身。需要更多的研究来更好地定义其临床方面和发病机制,并为这些疾病的诊断和治疗方法划定标准准则。
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[Paraneoplastic syndromes: pathogenetic theories, clinical aspects and therapeutic approach].

Paraneoplastic syndromes are uncommon diseases with different pathogenesis and clinical manifestations, correlated with neoplasms but not due to the tumor, metastasis or other distant effects. The aim of the present article is to describe the main paraneoplastic syndromes (neurological, endocrine-metabolic, rheumatological, osteo-articular, dermatological, hematological, vascular and nephrological), the associated pathogenetic theories (theory of the common embryonal sketches, theory of reactivation of the information and autoimmune theory) and the most important therapeutic approaches, on the basis of the literature. Experimental works, reviews and clinical observations, in some cases still in progress, regarding the described syndromes, their pathogenesis and their therapeutic approach have been examined. No meta-analyses regarding paraneoplastic syndromes have been published in the literature. The better described pathogenesis is the autoimmune one, characteristic of neurological, nephrologic and some dermatologic syndromes, for which the clinical and laboratory findings have been well supported. The pathogenetic theories associated with the other syndromes have been correlated on the basis of the literature. Paraneoplastic syndromes are important because their identification permits an early diagnosis of tumors and rapid treatment, with a largely improved prognosis and life expectancy for the patient. They often represent the only signal of a silent neoplasm; sometimes they precede the tumor itself. More studies are necessary for a better definition of their clinical aspects and pathogenesis and to delineate standard guidelines for a diagnostic-therapeutic approach to these diseases.

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