Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Syndrome Treated With Sirolimus
Authors: Maud Russier, Laurent Plantier, Gaelle Derot, Anne de Muret, Sylvain Marchand-Adam
Journal: Annals of Internal Medicine (2018)

Letters7 August 2018Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Syndrome Treated With SirolimusMaud Russier, MD, Laurent Plantier, MD, PhD, Gaelle Derot, MD, Anne de Muret, MD, and Sylvain Marchand-Adam, MD, PhDMaud Russier, MDCentre Hospitalier Régional d'Orléans, Orléans, France (M.R.)Search for more papers by this author, Laurent Plantier, MD, PhDUniversité François Rabelais, Tours, France (L.P., S.M.)Search for more papers by this author, Gaelle Derot, MDCentre Hospitalier Régional Universitaire de Tours, Tours, France (G.D., A.D.)Search for more papers by this author, Anne de Muret, MDCentre Hospitalier Régional Universitaire de Tours, Tours, France (G.D., A.D.)Search for more papers by this author, and Sylvain Marchand-Adam, MD, PhDUniversité François Rabelais, Tours, France (L.P., S.M.)Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/L17-0634 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder characterized by proliferation of neuroendocrine cells in the bronchial wall with formation of tumorlets and carcinoid tumors. The term DIPNECH syndrome has been applied to a subgroup of patients with this disorder who have respiratory symptoms, airflow obstruction, mosaic attenuation with air trapping on chest imaging, and constrictive obliterative bronchiolitis (1).Objective: To describe successful treatment of DIPNECH syndrome with sirolimus.Case Report: Three women aged 52, 53, and 61 years presented with exertional dyspnea. All were nonsmokers. According to the modified Medical Research Council dyspnea scale, 1 ...References1. Rossi G, Cavazza A, Spagnolo P, Sverzellati N, Longo L, Jukna A, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome. Eur Respir J. 2016;47:1829-41. [PMID: 27076588] doi:10.1183/13993003.01954-2015 CrossrefMedlineGoogle Scholar2. Gorshtein A, Gross DJ, Barak D, Strenov Y, Refaeli Y, Shimon I, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and the associated lung neuroendocrine tumors: clinical experience with a rare entity. Cancer. 2012;118:612-9. [PMID: 21751183] doi:10.1002/cncr.26200 CrossrefMedlineGoogle Scholar3. Zhou H, Ge Y, Janssen B, Peterson A, Takei H, Haque A, et al. Double lung transplantation for diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. J Bronchology Interv Pulmonol. 2014;21:342-5. [PMID: 25321455] doi:10.1097/LBR.0000000000000098 CrossrefMedlineGoogle Scholar4. Yao JC, Fazio N, Singh S, Buzzoni R, Carnaghi C, Wolin E, et al; RAD001 in Advanced Neuroendocrine Tumours, Fourth Trial (RADIANT-4) Study Group. Everolimus for the treatment of advanced, non-functional neuroendocrine tumours of the lung or gastrointestinal tract (RADIANT-4): a randomised, placebo-controlled, phase 3 study. Lancet. 2016;387:968-77. [PMID: 26703889] doi:10.1016/S0140-6736(15)00817-X CrossrefMedlineGoogle Scholar5. Rossi G, Cavazza A, Graziano P, Papotti M. mTOR/p70S6K in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia [Letter]. Am J Respir Crit Care Med. 2012;185:341. [PMID: 22298367] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: Centre Hospitalier Régional d'Orléans, Orléans, France (M.R.)Université François Rabelais, Tours, France (L.P., S.M.)Centre Hospitalier Régional Universitaire de Tours, Tours, France (G.D., A.D.)Disclosures: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=L17-0634.This article was published at Annals.org on 10 April 2018. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia: DIPNECHConstrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasiaUn cas clinique de syndrome d’hyperplasie neuroendocrine pulmonaire diffuse idiopathique (DIPNECH)Recent advances in the understanding of bronchiolitis in adultsSirolimus 7 August 2018Volume 169, Issue 3Page: 197-198KeywordsBronchodilatorsCarcinoid tumorCell proliferationComputed axial tomographyDyspneaForced expiratory volume in the first second of expirationLung and intrathoracic tumorsLungsSomatostatinThorax ePublished: 10 April 2018 Issue Published: 7 August 2018 Copyright & PermissionsCopyright © 2018 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...