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<p>Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) is a rare preneoplastic condition, frequently underdiagnosed due to its insidious course and non-specific symptoms that mimic common obstructive pulmonary diseases, such as asthma or chronic obstructive pulmonary disease (COPD). We report three clinical cases representing the primary phenotypes of this entity: (1) a patient with obstructive bronchial syndrome initially treated as chronic bronchitis; (2) an asymptomatic patient with an incidental imaging finding of multiple bilateral pulmonary nodules; and (3) a patient with ectopic Cushing syndrome secondary to a pulmonary neuroendocrine tumor arising from DIPNECH foci. The pathophysiology of DIPNECH involves pathways such as mTOR and the overexpression of somatostatin receptors (SSTR), triggering a morphological continuum that progresses from cellular hyperplasia to tumorlets and carcinoid tumors. Diagnosis requires a high index of clinical suspicion, recognition of characteristic CT patterns (diffuse nodules and mosaic attenuation), and histopathological confirmation. The treatment of DIPNECH must be individualized. Although its prognosis is generally favorable and indolent, a multidisciplinary approach is required for managing obstructive symptoms, monitoring neoplastic potential, and treating the emergence of endocrine syndromes.</p>

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The spectrum of DIPNECH: a review based on 3 cases

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年8月5日

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  • Alejando Pinzon-Tovar,
  • Giovani Lastra-González,
  • Rina Liliana Luna-Tavera,
  • Juan Diego Cruz-Rojas,
  • Laura Mercedes Bonelo-Celly

摘要

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) is a rare preneoplastic condition, frequently underdiagnosed due to its insidious course and non-specific symptoms that mimic common obstructive pulmonary diseases, such as asthma or chronic obstructive pulmonary disease (COPD). We report three clinical cases representing the primary phenotypes of this entity: (1) a patient with obstructive bronchial syndrome initially treated as chronic bronchitis; (2) an asymptomatic patient with an incidental imaging finding of multiple bilateral pulmonary nodules; and (3) a patient with ectopic Cushing syndrome secondary to a pulmonary neuroendocrine tumor arising from DIPNECH foci. The pathophysiology of DIPNECH involves pathways such as mTOR and the overexpression of somatostatin receptors (SSTR), triggering a morphological continuum that progresses from cellular hyperplasia to tumorlets and carcinoid tumors. Diagnosis requires a high index of clinical suspicion, recognition of characteristic CT patterns (diffuse nodules and mosaic attenuation), and histopathological confirmation. The treatment of DIPNECH must be individualized. Although its prognosis is generally favorable and indolent, a multidisciplinary approach is required for managing obstructive symptoms, monitoring neoplastic potential, and treating the emergence of endocrine syndromes.