title: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome.
abstract: The term diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) may be used to describe a clinico-pathological syndrome, as well as an incidental finding on histological examination, although there are obvious differences between these two scenarios. According to the World Health Organization, the definition of DIPNECH is purely histological. However, DIPNECH encompasses symptomatic patients with airway disease, as well as asymptomatic patients with neuroendocrine cell hyperplasia associated with multiple tumourlets/carcinoid tumours. DIPNECH is also considered a pre-neoplastic lesion in the spectrum of pulmonary neuroendocrine tumours, because it is commonly found in patients with peripheral carcinoid tumours.In this review, we summarise clinical, physiological, radiological and histological features of DIPNECH and critically discuss recently proposed diagnostic criteria. In addition, we propose that the term "DIPNECH syndrome" be used to indicate a sufficiently distinct patient subgroup characterised by respiratory symptoms, airflow obstruction, mosaic attenuation with air trapping on chest imaging and constrictive obliterative bronchiolitis, often with nodular proliferation of neuroendocrine cells with/without tumourlets/carcinoid tumours on histology. Surgical lung biopsy is the diagnostic gold standard. However, in the appropriate clinical and radiological setting, transbronchial lung biopsy may also allow a confident diagnosis of DIPNECH syndrome.
authors.affiliation: Section of Pathologic Anatomy, University Hospital Policlinico of Modena, Modena, Italy.
authors.forename: Giulio
authors.initials: G
authors.lastname: Rossi
authors.affiliation: Dept of Oncology and Advanced Technologies, Operative Unit of Oncology, Arcispedale S. Maria Nuova/I.R.C.C.S., Reggio Emilia, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Cavazza
authors.affiliation: Medical University Clinic, Canton Hospital Baselland and University of Basel, Basel, Switzerland Section of Respiratory Diseases, Dept of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy paolo.spagnolo@unipd.it.
authors.forename: Paolo
authors.initials: P
authors.lastname: Spagnolo
authors.affiliation: Section of Diagnostic Imaging, Dept of Surgery, University of Parma, Parma, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Sverzellati
authors.affiliation: Medical Oncology Unit, Civic Hospital "Ramazzini", Carpi, Italy.
authors.forename: Lucia
authors.initials: L
authors.lastname: Longo
authors.affiliation: Pathology Institute, Pauls Stradins Clinical University Hospital, Riga, Latvia.
authors.forename: Agita
authors.initials: A
authors.lastname: Jukna
authors.affiliation: Respiratory Disease Clinic, University Hospital Policlinico di Modena, Modena, Italy.
authors.forename: Gloria
authors.initials: G
authors.lastname: Montanari
authors.affiliation: Operative Unit of Pulmonology, Arcispedale S. Maria Nuova/I.R.C.C.S., Reggio Emilia, Italy.
authors.forename: Cristiano
authors.initials: C
authors.lastname: Carbonelli
authors.affiliation: Dept of Life Sciences, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Giada
authors.initials: G
authors.lastname: Vincenzi
authors.affiliation: Section of Pathologic Anatomy, Hospital "Don Calabria", Verona, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Bogina
authors.affiliation: Pathologic Anatomy, Istituto Nazionale Tumori Fondazione "Pascale", Naples, Italy.
authors.forename: Renato
authors.initials: R
authors.lastname: Franco
authors.affiliation: Division of Medical Oncology University Hospital, Parma, Italy.
authors.forename: Marcello
authors.initials: M
authors.lastname: Tiseo
authors.affiliation: Hospices Civils de Lyon, Hôpital Louis Pradel, National Reference Center for Rare Pulmonary Diseases, Lyon, France Claude Bernard Lyon 1 University, University of Lyon, Lyon, France INRA, UMR754, Lyon, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.affiliation: Dept of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA.
authors.forename: Thomas V
authors.initials: TV
authors.lastname: Colby
mesh_terms: D001706:Biopsy; D002276:Carcinoid Tumor / Q000503:physiopathology; D049109:Cell Proliferation; D003937:Diagnosis, Differential; D006801:Humans; D006965:Hyperplasia / Q000503:physiopathology*; D007150:Immunohistochemistry; D008168:Lung / Q000503:physiopathology; D008175:Lung Neoplasms / Q000473:pathology; D055613:Multiple Pulmonary Nodules / Q000175:diagnosis* / Q000503:physiopathology*; D055099:Neuroendocrine Cells / Q000473:pathology*; D011230:Precancerous Conditions / Q000503:physiopathology; D011658:Pulmonary Fibrosis / Q000503:physiopathology; D012119:Respiration; D013577:Syndrome