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

Author:

Samhouri Bilal F12ORCID,Halfdanarson Thorvardur R3ORCID,Koo Chi Wan4,McCarthy Cormac56,Yi Eunhee S7,Thomas Charles F1,Ryu Jay H1

Affiliation:

1. Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA

2. Division of Pulmonary and Critical Care Medicine, Asante Health System, Medford, Oregon, USA

3. Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA

4. Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA

5. School of Medicine, University College Dublin, Dublin, Ireland

6. Department of Respiratory Medicine, St Vincent’s University Hospital, Elm Park, Dublin, Ireland

7. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare, but increasingly recognized entity that primarily affects middle-aged and elderly women. It is characterized by abnormal proliferation of pulmonary neuroendocrine cells (PNECs) and is considered a preinvasive lesion for carcinoid tumorlets/tumors. Sometimes, DIPNECH is accompanied by constrictive bronchiolitis which usually manifests as chronic cough and/or dyspnea, along with airflow limitation on spirometry. The telltale imaging sign of DIPNECH is the presence of multiple noncalcified pulmonary nodules and mosaic attenuation on CT. However, these clinico-radiologic features of DIPNECH are characteristic but nonspecific; thus, histopathologic confirmation is usually necessary. DIPNECH has an indolent course and only rarely leads to respiratory failure or death; progression to overt neuroendocrine tumor (carcinoid) of the lung occurs in a minority of patients. Of available therapies, somatostatin analogs and mechanistic target of rapamycin inhibitors are the most promising. In this review, we provide an update regarding the diagnosis and management of DIPNECH and describe critical gaps in our understanding of this entity, including the central terms ‘diffuse’ and ‘idiopathic.’ We also summarize the inconsistencies in definitions employed by recent studies and discuss the pitfalls of the DIPNECH definitions proposed by the World Health Organization in 2021. In this context, we propose an objective and reproducible radio-pathologic case definition intended for implementation in the research realm and seeks to enhance homogeneity across cohorts. Furthermore, we discuss aspects of PNECs biology which suggest that PNEC hyperplasia may contribute to the pathogenesis of phenotypes of lung disease aside from constrictive bronchiolitis and carcinoid tumorlets/tumors. Finally, we steer attention to some of the most pressing and impactful research questions awaiting to be unraveled.

Publisher

Bioscientifica

Subject

Cancer Research,Endocrinology,Oncology,Endocrinology, Diabetes and Metabolism

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