Purdy Adam, Ido Firas, Stahlnecker Deborah
Division of Pulmonary and Critical Care, St. Luke's University Health Network, Bethlehem, PA, USA.
Case Rep Pulmonol. 2021 Dec 10;2021:6312296. doi: 10.1155/2021/6312296. eCollection 2021.
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an atypical pulmonary disorder with limited understanding. Given the rare nature of this disease, it is essential to obtain adequate tissue pathology to confirm the diagnosis. This disease is mainly diagnosed in middle-aged, nonsmoking females, and it is now accepted as a precursor lesion to pulmonary carcinoid tumors. DIPNECH presents with characteristic radiographic and histologic findings, but its diagnosis, management, and prognosis are often underrecognized and poorly understood. Those with symptoms may present with shortness of breath, wheezing, and persistent cough and are often misdiagnosed with reactive airway disease. Pulmonary function testing may reveal airflow obstruction and air trapping. Imaging is characterized by multiple lung nodules, typically less than 5 mm in size, with a background mosaic attenuation on computed tomography imaging. Histologically, DIPNECH can be suspected based on the presence of hyperplastic neuroendocrine cells. DIPNECH is considered a precursor to invasive neuroendocrine tumor, and up to 50% of patients may have a well-differentiated neuroendocrine tumor at the time of presentation. Here, we present the case of a 46-year-old female with a history of ulcerative colitis on mesalamine who presented with a 6-month history of ongoing shortness of breath, chest tightness, wheezing, and cough. She was initially diagnosed with asthma before imaging later revealed as multiple pulmonary nodules with a diffuse mosaic pattern. Using robotic-assisted navigational bronchoscopy, she underwent sampling of a dominant 1.8 cm right middle lobe pulmonary nodule and pathology was consistent with low-grade neuroendocrine tumor.
弥漫性特发性肺神经内分泌细胞增生症(DIPNECH)是一种了解有限的非典型肺部疾病。鉴于该疾病的罕见性,获取足够的组织病理学检查以确诊至关重要。这种疾病主要在中年非吸烟女性中被诊断出来,现在被认为是肺类癌肿瘤的前驱病变。DIPNECH具有特征性的影像学和组织学表现,但其诊断、管理和预后常常未得到充分认识且了解不足。有症状的患者可能表现为呼吸急促、喘息和持续性咳嗽,常被误诊为反应性气道疾病。肺功能测试可能显示气流阻塞和气体潴留。影像学特征为多个肺结节,通常直径小于5毫米,在计算机断层扫描成像上伴有背景马赛克样衰减。在组织学上,根据增生的神经内分泌细胞的存在可怀疑为DIPNECH。DIPNECH被认为是侵袭性神经内分泌肿瘤的前驱病变,高达50%的患者在就诊时可能患有高分化神经内分泌肿瘤。在此,我们报告一例46岁患有溃疡性结肠炎且正在服用美沙拉嗪的女性患者,她有6个月持续呼吸急促、胸闷、喘息和咳嗽的病史。她最初被诊断为哮喘,后来影像学检查显示为多个伴有弥漫性马赛克样图案的肺结节。通过机器人辅助导航支气管镜检查,她对一个位于右中叶的1.8厘米优势肺结节进行了采样,病理结果与低级别神经内分泌肿瘤一致。