Prognostic Significance of Pulmonary Multifocal Neuroendocrine Proliferation With Typical Carcinoid

Abstract

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A case of multiple lung carcinoid tumors localized in the right lower lobe

“…Considering the possible development of bilateral lung and chronic respiratory failure, a right lower lobectomy might have been excessive. The development of high-grade neuroendocrine tumors, such as small cell lung cancer or large cell neuroendocrine carcinoma, from DIPNECH has not been reported to date, but increase in size of nodules and lymph-nodes or distant metastases after long term follow-up were reported [ [14] , [15] , [16] ]. We decided to follow the patient carefully with annual CT surveillance instead of performing additional surgery.…”

Updates on lung neuroendocrine neoplasm classification

“…Single or multiple carcinoids may also be present, 16 strongly supporting the notion that the spectrum of neuroendocrine cell lesions, from neuroendocrine cell hyperplasia to carcinoids, represent a multistep model of progression. The clinical impact of DIPNECH in association with carcinoids is controversial, although some data suggest that multifocal pulmonary neuroendocrine proliferations represent a relevant adverse prognostic factor in carcinoid tumours, being associated with a higher risk of lymph node spread and tumour relapse 17 . Lastly, DIPNECH should be distinguished from neuroendocrine cell hyperplasia and tumourlets secondary to chronic lung disease (e.g.…”

“…The clinical impact of DIPNECH in association with carcinoids is controversial, although some data suggest that multifocal pulmonary neuroendocrine proliferations represent a relevant adverse prognostic factor in carcinoid tumours, being associated with a higher risk of lymph node spread and tumour relapse. 17 Lastly, DIPNECH should be distinguished from neuroendocrine cell hyperplasia and tumourlets secondary to chronic lung disease (e.g. inflammation, granulomas, fibrosis or high altitude), as well as from localised reactive neuroendocrine cell hyperplasia associated with carcinoid tumours or other lung neoplasms.…”

DIPNECH: pragmatic approach, uncertainties, notable associations, and a proposal for an improved definition

“…This entity was initially termed 'idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells and airways disease' (Aguayo et al 1992). Whilst this initial nomenclature implies that 'airways disease' is essential to this entity, subsequent studies have proven otherwise insofar as constrictive bronchiolitis was absent in a large proportion of patients (Carr et al 2015, Hayes et al 2022, Tassi et al 2022.…”

“…Its telltale imaging sign is the presence of multiple bilateral small noncalcified pulmonary nodules on CT imaging. Because these features are nonspecific (Rossi et al 2016, Hayes et al 2022, Tassi et al 2022, coupled with the limited awareness of this entity among clinicians (Carr et al 2015) and radiologists (Little et al 2020), DIPNECH gets frequently mislabeled as asthma and/or chronic obstructive pulmonary disease (COPD) for many years before its diagnosis (Carr et al 2015, Little et al 2020, Hayes et al 2022, Hurabielle et al 2022, Samhouri & Ryu 2023b. Confirming DIPNECH typically requires surgical lung biopsy (SLB) (Carr et al 2015, Rossi et al 2016, Hayes et al 2022, which is often avoided given its invasiveness (Samhouri et al 2022).…”