Management of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review and a Single Center Experience

Lung
Zin W MyintLowell B Anthony

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition, characterized by diffuse proliferation of neuroendocrine cells in the respiratory epithelium. DIPNECH lesions are less than 5 mm in size and are limited to the basement membrane with no invasion. There is limited information regarding epidemiology, natural history of disease progression, or the management of this rare entity. We present the experience of a center with extensive expertise in neuroendocrine disease. A cohort of patients (N = 13) with DIPNECH treated and followed at our institution was identified. We describe the our approach to their care, our disease management and also provide a review of DIPNECH pathophysiology. Our patient cohort consisted of twelve females and one male with a mean age of 63 years at the time of diagnosis. Dyspnea on exertion and dry cough were the most common presenting symptoms. Two patients were under surveillance without treatment; three patients were treated with a short-acting somatostatin analog; three patients were treated with azithromycin alone; four were treated with a combination of long-acting monthly somatostatin analogs and azithromycin; one patient received a combination of lo...Continue Reading

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Citations

Oct 7, 2020·AJR. American Journal of Roentgenology·Brent P LittleEugene A Berkowitz
Feb 11, 2021·The New England Journal of Medicine·Richard M SchwartzsteinLida P Hariri
Mar 1, 2021·Current Treatment Options in Oncology·Taymeyah Al-ToubahJonathan Strosberg
May 1, 2021·Current Opinion in Pulmonary Medicine·Daniel R AlmquistMohamad Bassam Sonbol
Jul 3, 2021·Journal of Clinical Medicine·Cécile ChungGuillaume Chassagnon
Aug 6, 2021·Journal of Thoracic Oncology : Official Publication of the International Association for the Study of Lung Cancer·Medard F M van den BroekUNKNOWN DutchMEN Study Group
Apr 9, 2021·The Annals of Thoracic Surgery·Valentina TassiNiccolò Daddi

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