WCLC 2025 - Posters & ePosters-EP.13.47 The Subtle Signals of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): A Clinical Characterization

EP.13.47 The Subtle Signals of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): A Clinical Characterization

Back to course

Pdf Summary

This study focuses on Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH), a rare and underdiagnosed lung condition characterized by idiopathic proliferation of pulmonary neuroendocrine cells. DIPNECH is typically indolent but considered pre-malignant. Patients often present with respiratory symptoms such as dyspnea and cough. Radiographic findings frequently demonstrate mosaic attenuation and bilateral pulmonary nodules, while pulmonary function tests commonly reveal obstructive lung disease patterns.<br /><br />The researchers retrospectively analyzed 34 DIPNECH patients diagnosed through histologic or radiologic criteria across two academic centers. They documented patients' baseline characteristics and clinical outcomes, affirming the disease’s rarity and diagnostic challenges.<br /><br />Current treatment options include inhalers, somatostatin analogs, and the mTOR inhibitor everolimus, mainly targeting symptom control. Systemic therapies are generally reserved for patients experiencing significant symptomatic progression impacting quality of life. However, an optimal therapeutic approach remains undefined and requires further study.<br /><br />The authors emphasize the need for comprehensive, multidisciplinary management of DIPNECH patients due to the disease's complexity and the lack of highly effective treatments. Additionally, future research directions include exploring the connection between DIPNECH and the emergence of clinically significant carcinoid tumors, as well as identifying clinical predictors or biomarkers to guide therapy choices.<br /><br />In conclusion, DIPNECH remains an elusive diagnosis with limited treatment efficacy. Greater awareness, refined diagnostic criteria, and novel therapeutic strategies are essential to improve patient outcomes. This study underscores the importance of continued investigation into the pathophysiology and management of DIPNECH.

Asset Subtitle

Catherine Meador

Meta Tag

Speaker Catherine Meador

Topic Small Cell Lung Cancer and Neuroendocrine Tumors

Keywords

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia

DIPNECH

pulmonary neuroendocrine cells

pre-malignant lung disease

respiratory symptoms

mosaic attenuation

pulmonary nodules

obstructive lung disease

somatostatin analogs

everolimus