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Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome.

  • Rossi G Section of Pathologic Anatomy, University Hospital Policlinico of Modena, Modena, Italy.
  • Cavazza A Dept of Oncology and Advanced Technologies, Operative Unit of Oncology, Arcispedale S. Maria Nuova/I.R.C.C.S., Reggio Emilia, Italy.
  • Spagnolo P 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.
  • Sverzellati N Section of Diagnostic Imaging, Dept of Surgery, University of Parma, Parma, Italy.
  • Longo L Medical Oncology Unit, Civic Hospital "Ramazzini", Carpi, Italy.
  • Jukna A Pathology Institute, Pauls Stradins Clinical University Hospital, Riga, Latvia.
  • Montanari G Respiratory Disease Clinic, University Hospital Policlinico di Modena, Modena, Italy.
  • Carbonelli C Operative Unit of Pulmonology, Arcispedale S. Maria Nuova/I.R.C.C.S., Reggio Emilia, Italy.
  • Vincenzi G Dept of Life Sciences, University of Modena and Reggio Emilia, Modena, Italy.
  • Bogina G Section of Pathologic Anatomy, Hospital "Don Calabria", Verona, Italy.
  • Franco R Pathologic Anatomy, Istituto Nazionale Tumori Fondazione "Pascale", Naples, Italy.
  • Tiseo M Division of Medical Oncology University Hospital, Parma, Italy.
  • Cottin V 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.
  • Colby TV Dept of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA.
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  • 2016-04-15
Published in:
  • The European respiratory journal. - 2016
Biopsy
Carcinoid Tumor
Cell Proliferation
Diagnosis, Differential
Humans
Hyperplasia
Immunohistochemistry
Lung
Lung Neoplasms
Multiple Pulmonary Nodules
Neuroendocrine Cells
Precancerous Conditions
Pulmonary Fibrosis
Respiration
Syndrome
English 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.
Language
  • English
Open access status
bronze
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Persistent URL
https://sonar.ch/global/documents/49489
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