RESUMO
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare but important condition to consider when investigating a patient with suspected thoracic malignancy. There is very little known about DIPNECH and it is considered to be a precursor to carcinoid tumour of the lung. This case report aims to increase awareness of this largely unknown and rare condition and to better improve its consideration as a differential diagnosis in patients who remain unresponsive to conventional treatment.
Assuntos
Tumor Carcinoide , Pneumopatias , Neoplasias Pulmonares , Células Neuroendócrinas , Humanos , Células Neuroendócrinas/patologia , Hiperplasia/patologia , Pneumopatias/diagnóstico por imagem , Pneumopatias/etiologia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Tumor Carcinoide/diagnóstico , Tumor Carcinoide/diagnóstico por imagem , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/diagnóstico por imagemRESUMO
Introduction: Central airway obstruction (CAO) is a life-threatening complication of lung cancer. The prevalence of CAO in lung cancer patients is unknown. We audited CAO burden to inform our local cancer service. Methods: This is a cohort review of all new lung cancer diagnoses between 1 November 2014 and 30 November 2015. CAO was defined by CT appearance. CT scans and routine patient records were followed up to 30 November 2018 to determine the prevalence of CAO at diagnosis; the characteristics of patients with prevalent CAO; mortality (using survival analysis); and incident CAO over follow-up. Results: Of 342 new lung cancer diagnoses, CAO prevalence was 13% (95% CI 10% to 17%; n=45/342). Dedicated CT scan review identified missed CAO in 14/45 (31%) cases. In patients with prevalent CAO, 27/44 (61%) had a performance status of ≤2, 23/45 (51%) were diagnosed during an acute admission and 36/44 (82%) reported symptoms. Treatments were offered to 32/45 (71%); therapeutic bronchoscopy was performed in only 8/31 (26%) eligible patients. Median survival of patients with prevalent CAO was 94 (IQR 33-274) days. Multivariate analysis, adjusting for age, gender and disease stage, found CAO on index CT scan was independently associated with an increased hazard of death (adjusted HR 1.78 (95% CI 1.27 to 2.48); p=0.001). In total, 15/297 (5%) developed CAO during follow-up (median onset 340 (IQR 114-551) days). Over the audit period, 60/342 (18%; 95% CI 14% to 22%) had or developed CAO. Discussions: This is the first description of CAO prevalence in 40 years. Patients with prevalent CAO had a higher mortality. Our data provide a benchmark for service planning.