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Review
. 2022 Jan 20;14(3):520.
doi: 10.3390/cancers14030520.

Bronchial Carcinoids: From Molecular Background to Treatment Approach

Marta Araujo-Castro  1   2   3 , Eider Pascual-Corrales  1   2 , Javier Molina-Cerrillo  2   3   4 , Nicolás Moreno Mata  5 , Teresa Alonso-Gordoa  2   3   4
Affiliations
Review

Bronchial Carcinoids: From Molecular Background to Treatment Approach

Marta Araujo-Castro et al. Cancers (Basel). .

Abstract

A better understanding of the genetic and molecular background of bronchial carcinoids (BCs) would allow a better estimation of the risk of disease progression and the personalization of treatment in cases of advanced disease. Molecular studies confirmed that lungs neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) are different entities; thus, no progression of NET to NEC is expected. In BCs, MEN1 gene mutations and deletions and decreased gene expression have been associated with a poor prognosis. ATRX mutation has also been linked to a shorter disease-specific survival. In terms of therapeutic targets, PI3K/AKT/mTOR pathway mutations have been described in 13% of typical carcinoids (TCs) and 39% of atypical carcinoids (ACs), representing a targetable mutation with kinase inhibitors. Regarding treatment, surgical resection is usually curative in localized BCs and adjuvant treatment is not routinely recommended. Multiple options for systemic therapy exist for patients with advanced BCs, although limited by a heterogeneity in the scientific evidence behind their use recommendation. These options include somatostatin analogues, everolimus, peptide receptor radionuclide therapy, chemotherapy, radiotherapy, antiangiogenic agents, and immunotherapy. In this article, we provide a comprehensive review about the molecular and genetic background of BCs, and about the treatment of local and metastatic disease, as well as the main paraneoplastic syndromes that have been associated with this tumor.

Keywords: bronchial carcinoid; everolimus; lung neuroendocrine tumors; multiple endocrine neoplasia type 1; somatostatin analogues.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Overview of the most relevant molecular and genetic characteristics of bronchial carcinoids. ATRX: alpha thalassemia/mental retardation syndrome X-linked; LOH: loss of heterozygosity; RASSF1A: Ras association domain-containing protein 1; PIK3: phosphatidylinositol cinasa-3.
Figure 2
Figure 2
Treatment algorithm for advance lung neuroendocrine neoplasm. There is an unmet need for the classification of patients with a proliferative well differentiated NET with more than 10 mitosis/HPF.
Figure 3
Figure 3
Associated clinical syndromes in bronchial carcinoids: clinical manifestation and treatment options.
See this image and copyright information in PMC

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