Low Grade Neuroendocrine Tumors of the Lung

Abstract

The lung is the second most common site of neuroendocrine tumors (NETs). Typical and atypical carcinoids are low-grade NETs of the lung. They present a favorable prognosis comported to the more common high-grade NETs. The low- and high-grade NETs require different treatment strategies; effective management of these tumors is essential to prolong survival and to manage the symptoms in patients with secretory or functional tumors. These rare tumors have received little attention and education is needed for treating physicians. This mini-review will concentrate mainly on advanced low-grade lung NETs. The article describes the classification of lung NETs and the diagnostic work-up. Different treatment methods including somatostatin analogs, peptide receptor radioligand therapy, and biologic systemic therapy are discussed. Promising results from recent trials are presented and discussed in the context of the lung primary site.

Lung neuroendocrine tumors: A systematic literature review (Review)

“…Eloesser, using a transbronchial approach, performed the first surgical resection of a bronchial carcinoid in 1939 (7). The neuroendocrine cells from which neuroendocrine tumors (NETs) derive are located in numerous places in the human body (8). The lung is the second organ, after the liver, in the localization of NETs, making up 25% of all NETs and 1-2% of all cancers (9).…”

“…Typical carcinoid (TC) is 10 times more frequent than the atypical one that metastasizes in 50% of cases (19). There have been cases of double metachronous primary NETs (in the larynx and lung) reported in literature (7)(8)(9). In these cases, it is difficult to establish whether the tumors are metachronous or one of them is primary and the other is secondary.…”

Pulmonary Neuroendocrine Tumor Presenting as a Left Pleural Effusion

“…14 Pulmonary NETs may be functional and secrete these substances, leading to systemic symptoms based on the released molecules. 15 However, pulmonary NETs produce less serotonin than gastrointestinal NETs; therefore, carcinoid syndrome is less…”

Diagnostic and Management Pathways for Pulmonary Carcinoid Tumours in the United Kingdom: Results from the National Lung Neuroendocrine Tumour Pathway Project

“…Pulmonary carcinoid tumours (PC), a subset of pulmonary neuroendocrine tumours (NETs), are rare neuroendocrine epithelial malignancies [1,2]. PC are classified as well-differentiated low-grade (or grade 1) typical carcinoid tumours (TC) and poorly differentiated intermediate-grade (or grade 2) atypical carcinoid tumours (AC) [1][2][3][4][5]. Despite having a high overall survival rate, TCs metastasise in 10-15% of cases and ACs in up to 50% [6,7], with the potential for recurrence to occur many years after completion of primary treatment [8].…”