Alabi Fortune O, Alabi Christopher O, Alkhateeb Hadaya A, Fanaian Naim K, Lama Maximo E, Ghaneie Ashkan
Pulmonary and Critical Care Physician, Florida Lung Asthma and Sleep Specialists, Orlando, FL, USA.
Internal Medicine Intern and Research Associate, Florida Lung Asthma and Sleep Specialists, Orlando, FL, USA.
Respir Med Case Rep. 2020 Nov 12;31:101250. doi: 10.1016/j.rmcr.2020.101250. eCollection 2020.
Diffuse idiopathic pulmonary neuroendocrine cell (DIPNECH syndrome) remains unfamiliar to most clinicians even though it was first described almost 30 years ago. Diagnosis is usually confirmed histopathologically after lung biopsy, but often, a diagnosis or suspected diagnosis can be made radiographically. In this paper, we present a case report of a 68-year-old female with shortness of breath and fleeting pulmonary nodules observed on chest CT scan. She was initially misdiagnosed with asthma based on an abnormal pulmonary function test which revealed an obstructive ventilatory defect. The classic radiographic findings of DIPNECH syndrome and the typical patient demographics that should arouse suspicion of a DIPNECH diagnosis were also illustrated. DIPNECH syndrome is a clinicopathological syndrome whereas focal NECH is a pathological diagnosis that is often made incidentally on histological examination and is encountered in a variety of settings, including in resected carcinoid tumors, in the context of reactive changes concomitant with infection, in metastatic cancer, radiation pneumonitis, intra-lobar sequestration, smokers, interstitial lung disease, and lung adenocarcinoma. There are no proven treatments for DIPNECH syndrome. In patients with obstructive ventilatory symptoms, bronchodilators with inhaled steroids are usually prescribed. Some severe cases may require parenteral steroids. Somatostatin analogs (SSA) have also been used in some cases with mixed results. Rapamycin has been used in several cases based on the purported activation of the mammalian target of rapamycin (mTOR) in DIPNECH. Some patients with large carcinoid tumors may benefit from resection.
弥漫性特发性肺神经内分泌细胞增生症(DIPNECH综合征)尽管在近30年前就首次被描述,但大多数临床医生对其仍不熟悉。诊断通常在肺活检后通过组织病理学确认,但通常也可通过影像学做出诊断或疑似诊断。在本文中,我们报告了一例68岁女性病例,该患者有呼吸急促症状,胸部CT扫描发现有短暂性肺结节。她最初因肺功能测试异常显示阻塞性通气功能障碍而被误诊为哮喘。文中还阐述了DIPNECH综合征的典型影像学表现以及应引起对DIPNECH诊断怀疑的典型患者人口统计学特征。DIPNECH综合征是一种临床病理综合征,而局灶性神经内分泌细胞增生症是一种病理诊断,常在组织学检查时偶然发现,见于多种情况,包括在切除的类癌肿瘤中、在伴有感染的反应性改变情况下、在转移性癌、放射性肺炎、叶内型肺隔离症、吸烟者、间质性肺疾病及肺腺癌中。目前尚无经证实有效的DIPNECH综合征治疗方法。对于有阻塞性通气症状的患者,通常会开具吸入性类固醇的支气管扩张剂。一些严重病例可能需要胃肠外给予类固醇。生长抑素类似物(SSA)在某些病例中也有应用,但结果不一。基于DIPNECH中雷帕霉素哺乳动物靶点(mTOR)的所谓激活,雷帕霉素已在几例病例中使用。一些患有大的类癌肿瘤的患者可能从手术切除中获益。