Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome

Area of Science:

• Pulmonology
• Pathology
• Radiology

Background:

• Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is defined histologically by WHO but presents as a clinico-pathological syndrome or incidental finding.
• It is considered a pre-neoplastic lesion, often associated with peripheral carcinoid tumors.
• DIPNECH encompasses symptomatic airway disease and asymptomatic neuroendocrine cell hyperplasia with tumorlets/carcinoids.

Purpose of the Study:

• To review the clinical, physiological, radiological, and histological features of DIPNECH.
• To critically evaluate recently proposed diagnostic criteria for DIPNECH.
• To propose the term "DIPNECH syndrome" for a distinct subgroup of patients.

Main Methods:

• Review of clinical, physiological, radiological, and histological data.
• Critical discussion of diagnostic criteria.
• Analysis of patient subgroups based on symptoms and findings.

Main Results:

• DIPNECH syndrome is characterized by respiratory symptoms, airflow obstruction, mosaic attenuation with air trapping, and constrictive obliterative bronchiolitis.
• Histologically, it involves neuroendocrine cell proliferation with or without tumorlets/carcinoids.
• Surgical lung biopsy is the gold standard, but transbronchial biopsy may suffice in specific cases.

Conclusions:

• The term "DIPNECH syndrome" identifies a distinct patient group with characteristic respiratory and imaging findings.
• Accurate diagnosis requires integration of clinical, radiological, and histological data.
• Further research may refine diagnostic criteria and management strategies for DIPNECH syndrome.