Neuro-endocrine tumours of the lung. A review of relevant pathological and molecular data

Abstract

Neuroendocrine (NE) tumours of the lung include pure and mixed forms. In the former group, a continuum of lesions is recognised ranging from benign typical carcinoids to atypical carcinoids (having a low-grade behaviour, although often associated with regional and distant metastases), to the highly aggressive poorly differentiated carcinomas of the small and large cell types. In the mixed tumour group, the NE component is extensively represented in association with any of the non-small cell carcinoma subtypes (so-called combined carcinomas), or the NE component is restricted to a cell population scattered among adenocarcinoma cells (or more rarely within squamous or large cell carcinomas). The molecular profile of NE tumours has been widely investigated to identify features helpful for the diagnosis, prognosis and even therapy for this special lung tumour category. Specific chromosomal alterations, oncogene mutations and cell cycle molecule disregulation has been documented in NE tumours of the lung, as well as the expression of specific receptors or enzymes implicated in the response to biotherapies or to chemotherapeutic agents. The "molecular classification" of NE tumours should be integrated to morphology, for a better definition of the different histological types and a more appropriate selection of the therapeutic strategy.

DEK oncoprotein regulates transcriptional modifiers and sustains tumor initiation activity in high-grade neuroendocrine carcinoma of the lung

“…Unsupervised classification based on the copy number alterations showed a subtype of HGNEC with a better prognosis, suggesting the existence of molecular subtypes with clinical significance in HGNEC, but this observation should be validated in a further validation set. Multiple alterations in tumor suppressors and oncogenes have been reported (Sattler and Salgia, 2003;Righi et al, 2007), however, no prognostic DEK oncoprotein in pulmonary endocrine carcinoma T Shibata et al biomarker has been currently reported in HGNEC and our study has also failed to correlate our molecular classification with known oncoproteins. Therefore we further extracted specific loci that were associated with the prognostic classification to identify a novel diagnostic biomarker.…”

Goblet cell carcinoids and other mixed neuroendocrine/nonneuroendocrine neoplasms

“…According to the WHO classification of lung tumors [70] they are classified under the term "combined carcinomas". The morphology of such tumors, as well as their clinical behavior, recapitulate the specific features of the individual components, and this tumor group is described in detail elsewhere in the current issue [60].…”

Treatment of lung microcytoma with neuroendocrine differentiation in elderly patient

“…I tumori neuroendocrini (NET) del polmone non sono un'unica entità, ma costituiscono uno spettro di lesioni variamente differenziate associate a caratteristiche patologiche e comportamento clinico più o meno aggressivo. I carcinoidi tipici e atipici sono le varianti meglio differenziate, mentre le forme meno differenziate sono il carcinoma neuroendocrino a grandi cellule e il microcitoma [1,2].…”