Elsevier

Thoracic Surgery Clinics

Volume 24, Issue 3, August 2014, Pages 257-266
Thoracic Surgery Clinics

Pathology and Diagnosis of Neuroendocrine Tumors: Lung Neuroendocrine

https://doi.org/10.1016/j.thorsurg.2014.04.001Get rights and content

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Key points

  • Neuroendocrine tumors of the lung represent a spectrum of low-grade typical carcinoid (TC), intermediate-grade atypical carcinoid (AC), and high-grade large cell neuroendocrine carcinoma (LCNEC) and small cell lung carcinoma (SCLC).

  • The most important histologic feature used to distinguish the grade of lung NE tumors is the mitotic count.

  • Unlike in the gastrointestinal tract, Ki-67 is not established as a way to distinguish TC from AC. However, it is very useful for the separation of carcinoid

Introduction: nature of the problem

Neuroendocrine (NE) tumors of the lung range from the low-grade typical carcinoid (TC) and intermediate-grade atypical carcinoid (AC) to the high-grade large cell neuroendocrine carcinoma (LCNEC) and small cell lung carcinoma (SCLC) (Box 1).1, 2 SCLC is the most common NE lung tumor, representing approximately 14% of invasive lung cancers.3 LCNEC represents about 3% of lung cancers in surgical series. Carcinoid tumors account for 1% to 2% of invasive lung malignancies. AC are the rarest of the

Molecular Changes in Pulmonary Neuroendocrine Tumors

Recent molecular studies by the Clinical Lung Cancer Genome Project (CLCGP) clearly show that carcinoids show relatively few genetic changes when compared with the high-grade SCLC and LCNEC, supporting the concept in the 2004 World Health Organization (WHO) classification that these are 2 very different groups of tumors.5 Both SCLC and LCNEC frequently show mutations in TP53, RB1, and EP300. Additional genetic changes, such as copy number, can be found in some LCNEC that are characteristic of

Clinical features

SCLC is the most common pulmonary NE tumor, with more than 30,000 cases anticipated to be diagnosed in the United States in 2014.11 There has been a decrease in the frequency of SCLC cases over the past 30 years in the United States from 17% to 13% of all lung cancers, according to the United States National Cancer Institute Surveillance, Epidemiologic, and End Results (SEER) database.3, 12 Most patients present with advanced disease, but in approximately 5% of cases tumors can present as a

Summary

There are 4 major NE lung tumors, but the TC and AC tumors belong to a different family from the high-grade SCLC and LCNEC according to histologic, clinical, epidemiologic, and genetic features. Pathologically these tumors are primarily distinguished based on the mitotic counts, presence or absence of necrosis, and cytologic features (Fig. 6).

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