Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)-A Case Report

瀰漫性自發肺部神經內分泌細胞增生－病例報告

林書永(Shu-Yung Lin) ； 陳晉興(Jin-Shing Chen) ； 謝明書(Min-Shu Hsieh) ； 何肇基(Chao-Chi Ho)

《胸腔醫學》 29卷6期 (2014/12) Pp. 358-364

摘要

瀰漫性自發肺部神經內分泌細胞增生是一種罕見的肺部疾病，主要臨床表現為咳嗽、呼吸哮鳴聲、以及肺功能呈現阻塞性缺損。此疾病主要的致病機轉為肺部神經內分泌細胞瀰漫性增生或異生，產生多發的類癌微瘤、以及小支氣管周邊之纖維化造成小氣道之阻塞。我們報告一位七十歲病人，之前因哮鳴症狀被診斷為氣喘。在追蹤過程中因胸部X光出現左下結節而進一步檢查。電腦斷層顯現肺實質有拼貼狀變化，以及多顆肺部結節。手術切除肺部結節之後，病理診斷為瀰漫性自發肺部神經內分泌細胞增生。病人之症狀在使用吸入型類固醇及支氣管擴張劑下維持穩定，持續在門診追蹤。

關鍵字

瀰漫性自發肺部神經內分泌細胞增生 ； 神經內分泌細胞 ； 微瘤 ； 類癌 ； 肺部腫瘤

並列摘要

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition presenting with cough, wheezing, and obstructive ventilatory defect. The pathogenesis of the disease is diffuse hyperplasia or dysplasia of pulmonary neuroendocrine cells, multiple carcinoid tumorlets, and peribronchiolar fibrosis causing small airway obliteration. Herein, we present the case of a 70-year-old patient with this rare condition who had been treated for asthma for years, and who had a left lower lung tumor noted during follow-up chest radiography. Chest computed tomography showed a mosaic pattern of the lung parenchyma and multiple lung nodules. The pathological diagnosis of DIPNECH was made after a surgical resection of the lung nodules. The patient's symptoms remained stable with inhaled corticosteroid and bronchodilator use.

並列關鍵字

diffuse idiopathic pulmonary neuroendocrine cell hyperplasia ； neuroendocrine cells ； tumorlets ； carcinoid ； lung neoplasm