Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Gonda 18 South, Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA.
Department of Radiology, Mayo Clinic, Rochester, MN, USA.
Orphanet J Rare Dis. 2021 Nov 22;16(1):490. doi: 10.1186/s13023-021-02103-w.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is characterized by multifocal proliferation of pulmonary neuroendocrine cells. On chest CT, DIPNECH exhibits bilateral pulmonary nodules and mosaic attenuation in most patients. We sought to: (1) assess the specificity of this pattern (i.e., bilateral pulmonary nodules together with mosaic attenuation) for DIPNECH; (2) describe its differential diagnosis; and (3) identify the clinico-radiologic features that may help prioritize DIPNECH over other diagnostic considerations.
We searched the Mayo Clinic records from 2015 to 2019 for patients with bilateral pulmonary nodules and mosaic attenuation on CT who had a diagnostic lung biopsy. A thoracic radiologist reviewed all CT scans. Chi-square test was used for categorical variables, and odds ratios were utilized to measure the association between certain variables and DIPNECH.
Fifty-one patients met our inclusion criteria; 40 (78%) were females and 34 (67%) were never-smokers. Median age was 65 (interquartile range 55-73) years. Lung biopsy was surgical in 21 patients (41%), transbronchial in 17 (33%), and transthoracic in 12 (24%); explanted lungs were examined in 1 (2%). Metastatic/multifocal cancer was the most common diagnosis, and was found in 17 (33%) cases. Bronchiolitis was diagnosed in 12 patients (24%), interstitial lung disease in 10 (20%), and DIPNECH in 5 (10%). Previous diagnosis of an obstructive lung disease (odds ratio 15.8; P = 0.002), and peribronchial nodular distribution on CT (odds ratio 14.4; P = 0.006) were significantly correlated with DIPNECH. Although statistical significance was not reached, DIPNECH nodules were more likely to display solid attenuations (80% vs. 67%, P = 0.45), and were more numerous; > 10 nodules were seen in 80% of DIPNECH cases vs. 52% in others (P = 0.23). Because DIPNECH primarily affects women, we analyzed the women-only cohort and found similar results.
Various disorders can manifest the CT pattern of bilateral pulmonary nodules together with mosaic attenuation, and this combination is nonspecific for DIPNECH, which was found in only 10% of our cohort. Previous diagnosis of an obstructive lung disease, and peribronchial distribution of the nodules on CT increased the likelihood of DIPNECH vs. other diagnoses.
弥漫特发性肺神经内分泌细胞增生症（DIPNECH）的特征是肺神经内分泌细胞的多灶性增殖。在胸部 CT 上，DIPNECH 在大多数患者中表现为双侧肺结节和马赛克衰减。我们旨在：（1）评估这种模式（即双侧肺结节伴马赛克衰减）对 DIPNECH 的特异性；（2）描述其鉴别诊断；（3）确定有助于将 DIPNECH 置于其他诊断考虑因素之前的临床放射特征。
我们在梅奥诊所的记录中搜索了 2015 年至 2019 年间接受 CT 检查发现双侧肺结节和马赛克衰减且进行了诊断性肺活检的患者。一名胸部放射科医生对所有 CT 扫描进行了审查。使用卡方检验进行分类变量分析，使用优势比来衡量某些变量与 DIPNECH 之间的关联。
51 名患者符合纳入标准；40 名（78%）为女性，34 名（67%）为从不吸烟者。中位年龄为 65（四分位距 55-73）岁。21 名患者（41%）接受了手术肺活检，17 名（33%）接受了经支气管活检，12 名（24%）接受了经胸活检；1 名（2%）进行了肺移植。最常见的诊断是转移性/多灶性癌症，有 17 例（33%）。12 例（24%）诊断为细支气管炎，10 例（20%）诊断为间质性肺病，5 例（10%）诊断为 DIPNECH。既往诊断为阻塞性肺病（优势比 15.8；P=0.002）和 CT 上的支气管周围结节分布（优势比 14.4；P=0.006）与 DIPNECH 显著相关。尽管未达到统计学意义，但 DIPNECH 结节更有可能显示实性衰减（80%比 67%，P=0.45），且数量更多；DIPNECH 病例中超过 10 个结节占 80%，而其他病例中占 52%（P=0.23）。由于 DIPNECH 主要影响女性，我们分析了女性队列，发现了类似的结果。
各种疾病都可能表现出双侧肺结节伴马赛克衰减的 CT 模式，这种组合对 DIPNECH 没有特异性，在我们的队列中仅发现 10%。既往诊断为阻塞性肺病和 CT 上结节的支气管周围分布增加了 DIPNECH 与其他诊断的可能性。
