Ramirez Robert A, Cass Amanda S, Das Satya, Low See-Wei, Mehrad Mitra, Rickman Otis B, Scherer Philip M, Thomas Katharine E, Gillaspie Erin A
Division of Hematology and Oncology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
Transl Lung Cancer Res. 2022 Dec;11(12):2567-2587. doi: 10.21037/tlcr-22-415.
Low and intermediate grade neuroendocrine tumors of the lung are uncommon malignancies representing 2% of all lung cancers. These are termed typical and atypical pulmonary carcinoid tumors. These can arise in the setting of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). The presentation, workup, management and outcomes of patients with these tumors can overlap with more common lung cancers but differ in that many of these patients have a prolonged clinical course. The objective of this narrative review is to summarize the literature and provide evidence and expert-based algorithms for work up and treatment of pulmonary carcinoids and DIPNECH.
A search of PubMed and Web of Science databases ending April 15, 2022, with the following keywords "lung carcinoid", "DIPNECH", "lung neuroendocrine," and "bronchopulmonary carcinoid".
Pulmonary carcinoid tumors benefit from a multidisciplinary approach. Pre-treatment imaging with contrast-enhanced computed tomography, and DOTATATE positron emission tomography is required. Surgical resection is the gold standard for curative intent, and possibly including sublobar resections. Patients can recur or develop new primaries thus emphasizing the importance of surveillance; national guidelines recommend at least a 10-year follow up. A growing body of literature support the use of endobronchial therapy, with long responses documented. Systemic therapy consists of everolimus, somatostatin analogs, peptide receptor radionuclide therapy, and chemotherapy. Diffuse idiopathic pulmonary neuroendocrine tumor cell hyperplasia is rare, but series suggest somatostatin analogs may confer clinical benefit.
Pulmonary carcinoid tumors and DIPNECH are rare. Despite lack of regulatory approvals for advanced disease, multiple options are available but should be sequenced according to the clinical status and disease biology. Each patient should be discussed in a multidisciplinary setting and clinical trials should be considered if available.
肺低级别和中级别神经内分泌肿瘤是罕见的恶性肿瘤，占所有肺癌的2%。这些肿瘤被称为典型和非典型肺类癌肿瘤。它们可发生于弥漫性特发性肺神经内分泌细胞增生（DIPNECH）的背景下。这些肿瘤患者的临床表现、检查、治疗及预后可能与更常见的肺癌有重叠，但不同之处在于许多此类患者的临床病程较长。本叙述性综述的目的是总结文献，并为肺类癌和DIPNECH的检查及治疗提供基于证据和专家意见的算法。
检索截至2022年4月15日的PubMed和Web of Science数据库，关键词如下：“肺类癌”、“DIPNECH”、“肺神经内分泌”和“支气管肺类癌”。
肺类癌肿瘤受益于多学科方法。需要进行对比增强计算机断层扫描和DOTATATE正电子发射断层扫描的预处理成像。手术切除是根治性治疗的金标准，可能包括肺叶下切除。患者可能复发或出现新的原发肿瘤，因此强调了监测的重要性；国家指南建议至少进行10年的随访。越来越多的文献支持使用支气管内治疗，并有长期缓解的记录。全身治疗包括依维莫司、生长抑素类似物、肽受体放射性核素治疗和化疗。弥漫性特发性肺神经内分泌肿瘤细胞增生罕见，但系列研究表明生长抑素类似物可能带来临床益处。
肺类癌肿瘤和DIPNECH罕见。尽管晚期疾病缺乏监管批准，但有多种选择可用，但应根据临床状况和疾病生物学进行排序。每个患者都应在多学科环境中进行讨论，如有可用的临床试验也应考虑。
