Tsoli Marina, Dimitriadis Georgios K., Androulakis Ioannis I., Kaltsas Gregory, Grossman Ashley
1st Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens, Greece, Agiou Thoma 17, 11527, Athens, Greece
Department of Endocrinology and Metabolic Medicine, King's College Hospital NHS Foundation Trust, London, United Kingdom
Neuroendocrine neoplasms (NENs) are rare tumors that display marked heterogeneity with varying natural history, biological behavior, response to therapy and prognosis. Their management is complex, particularly as some may be associated with a secretory syndrome, and is undertaken in the context of a multidisciplinary team including a variety of surgical and medical options. The term paraneoplastic syndrome (PNS) is used to define a spectrum of symptoms attributed to the production of biologically active substances secreted from tumors not related to their specific organ or tissue of origin and/or production of autoantibodies against tumor cells. The majority of these syndromes is associated with hormonal and neurological symptoms. Currently, no specific underlying pathogenic mechanism has been identified although a number of plausible hypotheses have been put forward. PNSs can precede, occur concomitantly or present at a later stage of tumor development and may complicate the patient’s clinical course, response to treatment, and impact overall prognosis. Their detection can facilitate the diagnosis of the underlying neoplasia, monitor response to treatment, detect early recurrences, and correlate with prognosis. Clinical awareness and the incorporation into clinical practice of Ga-labelled somatostatin analogue positron emission tomography, and other evolving biomarkers have substantially contributed to the identification of patients harboring such syndromes. When associated with tumors of low malignant potential PNSs usually do not affect long-term outcome. Conversely, in cases of highly malignant tumors, endocrine PNSs are usually associated with poorer survival outcomes. The development of well-designed prospective multicenter trials remains a priority in the field in order to fully characterize these syndromes and provide evidence-based diagnostic and therapeutic protocols. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.
神经内分泌肿瘤（NENs）是罕见肿瘤，具有显著的异质性，其自然史、生物学行为、对治疗的反应和预后各不相同。其管理较为复杂，尤其是因为有些可能与分泌综合征相关，且需要在多学科团队的背景下进行，包括多种手术和医疗选择。副肿瘤综合征（PNS）一词用于定义一系列症状，这些症状归因于肿瘤分泌的生物活性物质的产生，而这些肿瘤与其特定的起源器官或组织无关，和/或归因于针对肿瘤细胞的自身抗体的产生。这些综合征大多与激素和神经症状相关。目前，虽然已经提出了一些合理的假设，但尚未确定具体的潜在致病机制。PNS可在肿瘤发展的前期、同时或后期出现，可能使患者的临床病程、对治疗的反应复杂化，并影响总体预后。其检测有助于潜在肿瘤的诊断、监测治疗反应、检测早期复发，并与预后相关。临床意识以及将镓标记的生长抑素类似物正电子发射断层扫描和其他不断发展的生物标志物纳入临床实践，极大地有助于识别患有此类综合征的患者。当与低恶性潜能肿瘤相关时，PNS通常不影响长期预后。相反，在高恶性肿瘤病例中，内分泌PNS通常与较差的生存结果相关。开展精心设计的前瞻性多中心试验仍然是该领域的首要任务，以便全面描述这些综合征，并提供基于证据的诊断和治疗方案。欲全面涵盖内分泌学的所有相关领域，请访问我们的在线免费网络文本，网址为WWW.ENDOTEXT.ORG。
