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Vol.59 No.1 contents | Japanese/English |
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- Invited Review Article -
Neuroendocrine Tumor of Lung and Mediastinum: the Updated Pathological Diagnosis and Treatment
Yasushi Goto1, Noriko Motoi21Department of Thoracic Oncology, 2Department of Pathology and Clinical Laboratories, National Cancer Center Hospital, Japan
Neuroendocrine tumors (NETs) of the lung and mediastinum are very rare, with annual incidences of only 1.49 and 0.02 per 100,000, respectively, according to Surveillance, Epidemiology, and End Results (SEER) data. According to the 4th edition of the WHO Classification of Tumours of the Lung, Pleura, Thymus and Heart, published in 2015, NETs of the lung and mediastinum include small cell carcinoma, large cell neuroendocrine carcinoma (LCNEC), and atypical and typical carcinoid tumor. Small cell carcinoma and large cell neuroendocrine carcinoma are considered high-grade lesions, atypical carcinoid as intermediate-grade lesions, and typical carcinoid as low-grade lesions. Of note, in the 4th edition WHO classification, NETs were introduced as a single major category, despite having been grouped into different categories in the previous (3rd) edition. The pathological diagnosis of LCNEC requires not only morphological characteristics but also the differentiation phenotype; this is a drastic change of mode. It is expected to help improve our understanding of a lesion's biological characteristics and facilitate the suitable selection of treatment options. The first-line treatment for pulmonary or mediastinal neuroendocrine tumors is surgical resection, but for unresectable cases, drug therapy is performed. For high-grade tumors, platinum-based chemotherapy is used, and for carcinoids, a wide range of treatment options, such as a somatostatin analogue and the mammalian target of rapamycin (mTOR) inhibitor everolimus, which is a molecular-targeted drug, are available. This review article outlines the epidemiology, pathological diagnosis, and treatment of pulmonary and mediastinal neuroendocrine tumors, including the latest findings. We hope this article will serve as a guide in routine clinical practice for rare tumors.
key words: Neuroendocrine tumor, Lung, Mediastinum, WHO pathological classification, Molecular target therapy
JJLC 59 (1): 37-45, 2019
