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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)
Melissa L. Rosado de Christenson, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)
                • Neuroendocrine cell (NEC)
              • Definitions

                • NEC: Normal component of respiratory epithelium
                  • Ability to synthesize, store, and secrete neuroamines and neuropeptides
                  • Postulated to have chemoreceptor role (e.g., detection of tissue hypoxia)
                • Neuroendocrine body: Tiny NEC aggregate
                • NEC hyperplasia (NECH): NEC proliferation in bronchial/bronchiolar epithelium
                  • DIPNECH: NEC proliferation associated with constrictive bronchiolitis, tumorlets, and carcinoids
                  • Reactive NECH: Associated with chronic lung disease, may occur in response to lung injury and hypoxia
                  • NECH near carcinoid tumors; incidental histologic finding
                • DIPNECH syndrome: Proposed term for symptomatic NEC proliferations and clinical constrictive bronchiolitis

              IMAGING

              • Radiographic Findings

                • CT Findings

                  • HRCT

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      Selected References

                                      1. Carr LL et al: Diffuse Idiopathic pulmonary neuroendocrine cell hyperplasia and neuroendocrine hyperplasia of infancy. Clin Chest Med. 37(3):579-87, 2016
                                      2. Rossi G et al: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome. Eur Respir J. 47(6):1829-41, 2016
                                      3. Benson RE et al: Spectrum of pulmonary neuroendocrine proliferations and neoplasms. Radiographics. 33(6):1631-49, 2013
                                      4. Klebe S et al: Facts and fiction: premalignant lesions of lung tissues. Pathology. 45(3):305-15, 2013
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)
                                                    • Neuroendocrine cell (NEC)
                                                  • Definitions

                                                    • NEC: Normal component of respiratory epithelium
                                                      • Ability to synthesize, store, and secrete neuroamines and neuropeptides
                                                      • Postulated to have chemoreceptor role (e.g., detection of tissue hypoxia)
                                                    • Neuroendocrine body: Tiny NEC aggregate
                                                    • NEC hyperplasia (NECH): NEC proliferation in bronchial/bronchiolar epithelium
                                                      • DIPNECH: NEC proliferation associated with constrictive bronchiolitis, tumorlets, and carcinoids
                                                      • Reactive NECH: Associated with chronic lung disease, may occur in response to lung injury and hypoxia
                                                      • NECH near carcinoid tumors; incidental histologic finding
                                                    • DIPNECH syndrome: Proposed term for symptomatic NEC proliferations and clinical constrictive bronchiolitis

                                                  IMAGING

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • HRCT

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Microscopic Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Consider

                                                                          Selected References

                                                                          1. Carr LL et al: Diffuse Idiopathic pulmonary neuroendocrine cell hyperplasia and neuroendocrine hyperplasia of infancy. Clin Chest Med. 37(3):579-87, 2016
                                                                          2. Rossi G et al: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome. Eur Respir J. 47(6):1829-41, 2016
                                                                          3. Benson RE et al: Spectrum of pulmonary neuroendocrine proliferations and neoplasms. Radiographics. 33(6):1631-49, 2013
                                                                          4. Klebe S et al: Facts and fiction: premalignant lesions of lung tissues. Pathology. 45(3):305-15, 2013