title: Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence.
abstract: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is recognized as a preneoplastic condition by the World Health Organization. We reviewed our experience with 30 patients and performed a systematic review of the English literature to collect best evidence on the clinical features and disease course in 169 additional patients. Some patients presented with one or more carcinoid tumors associated with multiple small pulmonary nodules on imaging studies and showed DIPNECH as a somewhat unexpected pathologic finding. Others presented with multiple small pulmonary nodules that raised suspicion of metastatic disease on imaging. A third subset was presented with previously unexplained respiratory symptoms. In most patients, DIPNECH was associated with a good prognosis, with chronological progression into a subsequent carcinoid tumor noted in only one patient and death attributed directly to DIPNECH in only two patients. There is no best evidence to support the use of octreotide, steroids, or bronchodilators in DIPNECH patients.
authors.affiliation: Department of Medicine, Cedars-Sinai Medical Center, 8700 Beverly Blvd Room 5512, West Hollywood, CA, 90048-1804, USA. eric.wirtschafter@cshs.org.
authors.forename: Eric
authors.initials: E
authors.lastname: Wirtschafter
authors.affiliation: Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
authors.forename: Ann E
authors.initials: AE
authors.lastname: Walts
authors.affiliation: Department of Hematology and Oncology, University of California Los Angeles, David Geffen School of Medicine, Los Angeles, CA, USA.
authors.forename: Sandy T
authors.initials: ST
authors.lastname: Liu
authors.affiliation: Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
authors.forename: Alberto M
authors.initials: AM
authors.lastname: Marchevsky
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D002276:Carcinoid Tumor / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D003371:Cough / Q000209:etiology; D018450:Disease Progression; D004417:Dyspnea / Q000209:etiology; D005260:Female; D006801:Humans; D006965:Hyperplasia / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D008168:Lung / Q000473:pathology*; D008171:Lung Diseases / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D008175:Lung Neoplasms / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D055613:Multiple Pulmonary Nodules / Q000000981:diagnostic imaging / Q000209:etiology / Q000628:therapy; D055099:Neuroendocrine Cells / Q000473:pathology*; D011013:Pneumonectomy; D011230:Precancerous Conditions / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D011859:Radiography; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D013004:Somatostatin / Q000031:analogs & derivatives / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D018931:Antineoplastic Agents, Hormonal; D013004:Somatostatin
keywords: Carcinoid tumor; DIPNECH; Diffuse idiopathic neuroendocrine cell hyperplasia; Preneoplasia; Tumorlet