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The ENETS/WHO grading system for neuroendocrine neoplasms of the gastroenteropancreatic system: a review of the current state, limitations and proposals for modifications. ENETS/世界卫生组织胃肠胰系统神经内分泌肿瘤分级系统：现状综述、限制和修改建议。
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-08-01 Epub Date: 2016-07-14 DOI: 10.2217/ije-2016-0006
Marcela S Cavalcanti, Mithat Gönen, David S Klimstra

The understanding of neuroendocrine neoplasms has evolved significantly since their initial descriptions in the 1800s to early 1900s. In the gastroenteropancreatic system, this group of malignant tumors is subdivided into well and poorly differentiated neuroendocrine neoplasms based on morphologic, proliferative and biologic differences. However, it has become increasingly apparent that well-differentiated neuroendocrine tumors are not a homogeneous group. Attempting to better predict outcome of these tumors has been the motivation behind numerous proposed classification systems, the evolution of which culminated with the currently used system, the ENETS/WHO classification. Herein, we review the genesis of this classification system and some of its shortcomings. In addition, we discuss some of the most recent proposals that suggest modifications to the current system.

自19世纪至20世纪初对神经内分泌肿瘤的最初描述以来，对其的理解发生了重大变化。在胃肠胰系统中，根据形态学、增殖性和生物学差异，这组恶性肿瘤可分为分化良好和分化较差的神经内分泌肿瘤。然而，越来越明显的是，分化良好的神经内分泌肿瘤并不是一个同质的群体。试图更好地预测这些肿瘤的结果一直是许多提议的分类系统背后的动机，这些分类系统的演变最终形成了目前使用的系统，即ENETS/世界卫生组织分类。在此，我们回顾了该分类系统的起源及其一些不足。此外，我们还讨论了一些建议修改现行制度的最新提案。
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引用次数: 27
Mahvash disease: an autosomal recessive hereditary pancreatic neuroendocrine tumor syndrome Mahvash病:常染色体隐性遗传胰腺神经内分泌肿瘤综合征
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-07-27 DOI: 10.2217/IJE-2016-0005
Jane Rhyu, R. Yu
Mahvash disease is a rare autosomal recessive hereditary pancreatic neuroendocrine tumor syndrome characterized by inactivating mutations in the glucagon receptor, hyperglucagonemia without symptoms of the glucagonoma syndrome, pancreatic α-cell hyperplasia and pancreatic neuroendocrine tumors. In Mahvash disease, the lack of negative feedback from glucagon receptor signaling results in compensatory hyperplasia of pancreatic α-cells and secondary tumorigenesis. Over ten clinical cases consistent with Mahvash disease have been reported thus far, most of which have confirmed biallelic mutations in the glucagon receptor. Mahvash disease provides insight into glucagon signaling pathway regulation and pathogenesis of pancreatic neuroendocrine tumors.
Mahvash病是一种罕见的常染色体隐性遗传性胰腺神经内分泌肿瘤综合征，其特征是胰高血糖素受体失活突变、无胰高血糖素综合征症状的高胰高血糖素血症、胰腺α-细胞增生和胰腺神经内分泌肿瘤。在Mahvash病中，缺乏胰高血糖素受体信号的负反馈导致胰腺α-细胞代偿性增生和继发性肿瘤发生。到目前为止，已经报道了十多例与Mahvash病一致的临床病例，其中大多数证实了胰高血糖素受体的双等位基因突变。Mahvash病提供了胰高血糖素信号通路调控和胰腺神经内分泌肿瘤的发病机制。
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引用次数: 4
Molecular diagnostics and anaplastic thyroid carcinoma: the time has come to harvest the high hanging fruit 分子诊断和间变性甲状腺癌:是时候收获高挂果实了
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-07-27 DOI: 10.2217/IJE-2016-0007
M. Cabanillas, N. Busaidy, S. Khan, G. Gunn, R. Dadu, S. Rao, S. Waguespack
Targeted therapies have played a major role in cancer therapeutics, starting with the discovery of a drug against BCR–ABL rearrangements in chronic myelogenous leukemia. This led to the first approval of a targeted agent in cancer and since, many others have followed. Anaplastic thyroid cancer (ATC) is an aggressive carcinoma with few curative options. Although previous cytotoxic chemotherapy and kinase inhibitor therapies have not proven efficacious in ATC, some of the newer drugs appear to be promising. A case report and a comprehensive review of the current standard of care, genetics, modern therapeutic drugs and clinical trials are presented, in order to outline where we currently stand and where the future lies in the quest for a cure for ATC.
靶向治疗在癌症治疗中发挥了重要作用，从发现一种针对慢性骨髓性白血病BCR-ABL重排的药物开始。这导致了癌症靶向药物的首次批准，此后，许多其他药物也相继获得批准。间变性甲状腺癌(ATC)是一种侵袭性癌症，治疗方法很少。虽然以前的细胞毒性化疗和激酶抑制剂治疗尚未被证明对ATC有效，但一些新药似乎很有希望。本文提出了一份病例报告，并对目前的护理标准、遗传学、现代治疗药物和临床试验进行了全面回顾，以概述我们目前的立场和未来寻求治疗ATC的方向。
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引用次数: 15
Thyroid incidentalomas and the overdiagnosis conundrum 甲状腺偶发瘤和过度诊断难题
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-07-14 DOI: 10.2217/IJE-2016-0008
R. Grogan, B. Aschebrook-Kilfoy, M. G. White, E. Kaplan, P. Angelos
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引用次数: 1
Should malnutrition screening be routine for patients with GEP-NET? 对GEP-NET患者是否应该进行常规营养不良筛查?
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-07-14 DOI: 10.2217/IJE-2016-0013
M. Weickert
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引用次数: 3
Epidemiological variations of aggressive growth hormone-secreting adenomas 侵袭性生长激素分泌腺瘤的流行病学变异
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-07-14 DOI: 10.2217/IJE-2015-0011
S. Vandeva, A. Elenkova, E. Natchev, S. Zacharieva
Acromegaly is a chronic disorder characterized by increased morbidity and mortality in uncontrolled patients. Growth hormone-secreting pituitary adenoma is the hallmark in the majority of cases, generally considered as benign due to lack of distant metastases. However, clinical behavior in a certain proportion of these adenomas could be quite aggressive, causing difficulties in their management. Aggressive pituitary adenomas have some clinical, radiological, ultrastructural and molecular features in common and they are usually resistant to the standard treatment. In the recent years, efforts have been made to define the most appropriate markers of such adenomas that would allow an early detection and efficient individualized therapeutic strategy. The aim of this review is to give an update on epidemiology and certain markers predicting aggressive behavior of somatotropinomas.
肢端肥大症是一种慢性疾病，其特点是在不受控制的患者中发病率和死亡率增加。生长激素分泌垂体腺瘤是大多数病例的标志，由于缺乏远处转移，通常被认为是良性的。然而，这些腺瘤中一定比例的临床表现可能相当具有侵袭性，给其治疗带来困难。侵袭性垂体腺瘤具有一些共同的临床、影像学、超微结构和分子特征，通常对标准治疗具有耐药性。近年来，人们一直在努力确定这种腺瘤的最合适的标记物，以便早期发现和有效的个性化治疗策略。这篇综述的目的是提供最新的流行病学和某些标志物预测生长激素瘤的攻击行为。
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引用次数: 2
Management of pancreatic neuroendocrine tumors 胰腺神经内分泌肿瘤的治疗
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-07-14 DOI: 10.2217/IJE-2016-0002
X. Keutgen, Bruna Babic, N. Nilubol
Pancreatic neuroendocrine tumors (pNETs) are rare tumors that have a better prognosis than their exocrine counterpart, but frequently present with advanced disease. Management of pNETs has evolved considerably over the past decade. Surgical resection remains the only potentially curative option for patients with pNETs. Patients who have locoregionally advanced and/or metastatic pNETs require additional treatments. These include liver-directed (transarterial (chemo)-embolization, selective intraarterial radio therapy) and systemic therapies (somatostatin analogs, targeted therapy such as tyrosine-kinase inhibitors and mammalian target of rapamycin inhibitor, peptide receptor radionuclide therapy and cytotoxic chemotherapy). The aim of this article is to review the current treatment options as well as potential future therapeutic perspectives for patients with pNETs.
胰腺神经内分泌肿瘤(pNETs)是一种罕见的肿瘤，其预后比外分泌肿瘤好，但经常出现晚期疾病。在过去十年中，pNETs的管理发生了很大的变化。手术切除仍然是pNETs患者唯一潜在的治疗选择。局部进展和/或转移性pNETs的患者需要额外的治疗。这些包括肝定向(经动脉化疗)栓塞，选择性动脉内放射治疗)和全身治疗(生长抑素类似物，酪氨酸激酶抑制剂和哺乳动物雷帕霉素抑制剂靶向治疗，肽受体放射性核素治疗和细胞毒性化疗)。本文的目的是回顾目前的治疗方案，以及潜在的未来治疗前景的pNETs患者。
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引用次数: 0
Initial surgical management of medullary thyroid cancer 甲状腺髓样癌的初步手术治疗
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-07-14 DOI: 10.2217/IJE-2015-0009
Kathryn E. Coan, Tracy S. Wang
Medullary thyroid cancer (MTC) accounts for 5–10% of thyroid cancer. The majority, 75–80%, of MTC are sporadic with the remainder being hereditary secondary to a mutation in the RET proto-oncogene. Hereditary MTC may be isolated as in familial medullary thyroid cancer or associated with multiple endocrine neoplasia syndrome types 2A and 2B. The primary treatment modality for sporadic MTC is total thyroidectomy and central compartment neck dissection; consideration of lateral neck dissection should be based on preoperative imaging findings. The timing of prophylactic thyroidectomy is dependent on the specific RET codon mutation.
甲状腺髓样癌(MTC)占甲状腺癌的5-10%。大多数MTC(75-80%)是散发性的，其余的是RET原癌基因突变的遗传性继发。遗传性甲状腺髓样癌可能与家族性甲状腺髓样癌分离，也可能与2A和2B型多发性内分泌瘤变综合征相关。散发性MTC的主要治疗方式是甲状腺全切除术和中央室颈清扫术;考虑侧颈剥离应基于术前影像学表现。预防性甲状腺切除术的时机取决于特定的RET密码子突变。
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引用次数: 0
Update on management of midgut neuroendocrine tumors. 中肠神经内分泌肿瘤的治疗进展。
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-05-01 Epub Date: 2016-04-08 DOI: 10.2217/ije-2015-0004
Amir Mehrvarz Sarshekeh, Daniel M Halperin, Arvind Dasari

Midgut neuroendocrine tumors are typically indolent but can be fatal when advanced. They can also cause significant morbidity due to the characteristic carcinoid syndrome. Somatostatin analogs continue to be the mainstay of treatment given their antiproliferative properties, as well as inhibitory effects on hormones that cause carcinoid syndrome. There have been several recent advances in the systemic therapy of these tumors including consolidation of somatostatin analogs as the cornerstone of therapy, completion of pivotal trials with mTOR inhibitors, and the establishment of novel approaches including peptide receptor radionuclide therapy and oral inhibitors of peripheral tryptophan hydroxylase in tumor and symptom control, respectively. In this review article, the recent advances are summarized and an updated approach to management is proposed.

中肠神经内分泌肿瘤通常是惰性的，但如果进展严重，可能是致命的。由于特征性的类癌综合征，它们也可引起显著的发病率。鉴于其抗增殖特性以及对引起类癌综合征的激素的抑制作用，生长抑素类似物仍然是治疗的主要手段。最近在这些肿瘤的全身治疗方面取得了一些进展，包括巩固生长抑素类似物作为治疗的基石，完成mTOR抑制剂的关键试验，以及建立新的方法，包括肽受体放射性核素治疗和口服外周色氨酸羟化酶抑制剂，分别用于肿瘤和症状控制。在这篇综述文章中，总结了最近的进展，并提出了一种更新的管理方法。
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引用次数: 6
Adrenocortical carcinoma: modern management and evolving treatment strategies. 肾上腺皮质癌:现代管理和不断发展的治疗策略。
IF 1.2
International Journal of Endocrine Oncology
Pub Date : 2016-05-01 Epub Date: 2016-04-08 DOI: 10.2217/ije-2015-0003
Lucas A McDuffie, Rachel D Aufforth
Adrenocortical carcinoma (ACC) is a rare cancer with a poor prognosis. Unlike many other cancers, there has been little improvement in patient outcome over the past several decades. However, as scientific advancements are made and our understanding of the molecular genetics involved in ACC improve then progress may be achieved in this devastating disease. This review focuses on recent literature published in the field of ACC from 2010 to 2015 with an emphasis on improving diagnosis, staging and treatment for ACC.
摘要肾上腺皮质癌(ACC)是一种罕见且预后差的癌症。与许多其他癌症不同，在过去的几十年里，患者的预后几乎没有改善。然而，随着科学的进步和我们对ACC分子遗传学的理解的提高，这种毁灭性疾病可能会取得进展。本文综述了2010年至2015年在ACC领域发表的最新文献，重点关注ACC的诊断、分期和治疗。
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