Girard Nicolas
University of Lyon, University Claude Bernard Lyon, Lyon, France.
Department of Respiratory Medicine, National Expert Centre for Thymic Malignancies, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France.
J Thorac Dis. 2017 Nov;9(Suppl 15):S1491-S1500. doi: 10.21037/jtd.2017.08.18.
Thymic malignancies represent a heterogeneous group of rare thoracic cancers, which are classified according to the World Health Organization (WHO) histopathologic classification that distinguishes thymomas from thymic carcinomas and neuroendocrine tumors; neuroendocrine thymic tumors (NETTs) exhibit the same histological spectrum as in other anatomical locations, although with different frequencies. NETTs represent around 2% of all neuroendocrine tumors, and about 5% of all thymic malignancies. Overall, the management of patients with NETTs tumors requires continuous multidisciplinary expertise at any step of the disease progression. Systemic treatment relies on cytotoxic chemotherapy, as well as on somatostatin analogues and everolimus. Systemic treatment may be delivered in a curative-intent approach, for patients presenting with locally-advanced tumor at the time of diagnosis, with invasion of intra-thoracic neighboring structures. In such cases, chemotherapy has been used in an induction setting, to reduce the tumor burden-possibly allowing subsequent surgery and/or radiotherapy-or as a postoperative treatment if the tumor was resectable upfront, to reduce the risk of recurrence and achieve prolonged disease control. Systemic therapies are also a palliative-intent treatment of unresectable, metastatic, and recurrent NETTs. Chemotherapy may then be an option for aggressive disease, but somatostatin analogues and everolimus are suitable as well. Alternative options are emerging through clinical trials. As no dedicated study has ever been conducted, recommendations for systemic treatment in NETTs have been mostly based on retrospective cohorts of limited numbers patients, especially in the advanced disease setting, and expert opinion based on experience from primary pulmonary, as well as gastro-intestinal neuroendocrine tumors, for which clinical trials have been conducted.
胸腺恶性肿瘤是一组异质性的罕见胸段癌症，根据世界卫生组织（WHO）的组织病理学分类进行区分，该分类将胸腺瘤与胸腺癌和神经内分泌肿瘤区分开来；神经内分泌胸腺肿瘤（NETTs）与其他解剖部位的肿瘤具有相同的组织学谱，尽管频率有所不同。NETTs约占所有神经内分泌肿瘤的2%，占所有胸腺恶性肿瘤的约5%。总体而言，NETTs肿瘤患者的管理在疾病进展的任何阶段都需要持续的多学科专业知识。全身治疗依赖于细胞毒性化疗以及生长抑素类似物和依维莫司。对于诊断时出现局部晚期肿瘤且侵犯胸内相邻结构的患者，全身治疗可以采用根治性治疗方法。在这种情况下，化疗已用于诱导治疗，以减轻肿瘤负荷，可能允许随后的手术和/或放疗；或者如果肿瘤术前可切除，则作为术后治疗，以降低复发风险并实现长期疾病控制。全身治疗也是不可切除、转移性和复发性NETTs的姑息性治疗。对于侵袭性疾病，化疗可能是一种选择，但生长抑素类似物和依维莫司也适用。通过临床试验正在出现其他选择。由于从未进行过专门研究，NETTs全身治疗的建议大多基于数量有限的患者回顾性队列，特别是在晚期疾病情况下，以及基于原发性肺和胃肠道神经内分泌肿瘤经验的专家意见，针对这些肿瘤已经进行了临床试验。
