The Surgical Management of Lung Neuroendocrine Neoplasms

Simple Summary Lung neuroendocrine neoplasms comprise a diverse group of cancers that arise from pulmonary neuroendocrine cells. They account for about 20% of all primary lung cancers and classify into four subtypes: typical carcinoid, atypical carcinoid, large cell neuroendocrine carcinoma, and small cell lung carcinoma. These subtypes share some morphological and protein expression immunohistochemistry features but differ greatly in their biological behaviors. Typical and atypical carcinoids are well differentiated low- and intermediate-grade tumors, respectively, whereas large cell neuroendocrine carcinoma and small cell lung carcinoma are poorly differentiated high-grade tumors. The common thread in all lung neuroendocrine neoplasms is that patients with localized disease primarily undergo surgery, while patients with locally advanced or metastatic disease receive tailored oncological therapies. This review summarizes key recent developments relevant to the surgical management of lung neuroendocrine neoplasms (L-NENs), including typical and atypical carcinoids, large cell neuroendocrine carcinoma, and small cell lung carcinoma. This review includes recent insights into the classification, clinical presentation, diagnostic workup, treatment options, and follow-up. Highlighted topics include general principles of surgery in localized or locally advanced or metastatic L-NENs, lung-sparing surgery for small, peripheral typical carcinoids, adjuvant and systemic therapies for typical and atypical carcinoids, and surgery and adjuvant therapies for large cell neuroendocrine carcinoma and small cell lung carcinoma.