title: DIPNECH: Association Between Histopathology and Clinical Presentation.
abstract: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder which can be an incidental finding in imaging tests performed during the investigation of another condition, or is the final diagnosis in patients evaluated for chronic obstructive complaints. To explore the possible association between specific histopathology features and the mode of clinical presentation, we retrieved the clinical, functional, radiological, and pathological data of all 13 patients diagnosed with DIPNECH at our Institution over a 14-year period (2000-2014). As compared to patients with incidental disease (6/13, 46 %), patients with symptomatic disease were younger [mean (SD): 57.7 vs. 68.7 years, p = 0.046], were more likely to have mosaic attenuation (100 vs. 0 %, p = 0.001) and small multiple nodules (100 vs. 17 %, p = 0.005) at CT, and showed a significantly higher number of foci of linear neuroendocrine proliferation [median (IQR): 28 (13-37) vs. 6 (5-13), p = 0.018] and of tumorlets [median (IQR): 10 (8-20) vs. 1 (1-1), p = 0.002] at histology. Incidental disease was found in association with pulmonary adenocarcinoma in five out of six patients (83.3 %). The results of our study provide preliminary evidence that symptomatic patients with DIPNECH represent a specific subset characterized by younger age and a higher burden of foci of neuroendocrine proliferation.
authors.affiliation: Interventional Pulmonology Unit, Policlinico Sant'Orsola-Malpighi, via Albertoni 15, 40138, Bologna, Italy. rocco.trisolini@aosp.bo.it.|Thoracic Endoscopy and Pulmonology Unit, Maggiore Hospital, Bologna, Italy. rocco.trisolini@aosp.bo.it.
authors.forename: Rocco
authors.initials: R
authors.lastname: Trisolini
authors.affiliation: Pulmonology Unit, City Hospital, Rimini, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Valentini
authors.affiliation: Clinical Epidemiology & Biometry Service, IRCCS Policlinico San Matteo, Pavia, Italy.
authors.forename: Carmine
authors.initials: C
authors.lastname: Tinelli
authors.affiliation: Pulmonology Unit, City Hospital, Rimini, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Ferrari
authors.affiliation: Thoracic Surgery Unit, Maggiore Hospital, Bologna, Italy.
authors.forename: Gian Marco
authors.initials: GM
authors.lastname: Guiducci
authors.affiliation: Thoracic Surgery Unit, Maggiore Hospital, Bologna, Italy.
authors.forename: Sergio Nicola Forti
authors.initials: SN
authors.lastname: Parri
authors.affiliation: Radiology Unit, Bellaria Hospital, Bologna, Italy.
authors.forename: Giorgia
authors.initials: G
authors.lastname: Dalpiaz
authors.affiliation: Pathology Unit, Maggiore Hospital, Bologna, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Cancellieri
mesh_terms: D000230:Adenocarcinoma / Q000150:complications / Q000473:pathology; D000077192:Adenocarcinoma of Lung; D000367:Age Factors; D000368:Aged; D058070:Asymptomatic Diseases; D001706:Biopsy; D049109:Cell Proliferation; D005260:Female; D006801:Humans; D006965:Hyperplasia; D007558:Italy; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D008171:Lung Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D008175:Lung Neoplasms / Q000150:complications / Q000473:pathology; D008297:Male; D008875:Middle Aged; D055099:Neuroendocrine Cells / Q000473:pathology*; D011379:Prognosis; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D013997:Time Factors; D014057:Tomography, X-Ray Computed
keywords: Bronchiolitis; Carcinoid; Computed tomography; Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia; Tumorlets