PDF(Pubmed)
Abstract：
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disease characterized by the diffuse proliferation of neuroendocrine cells in the bronchial epithelium. It is considered a preinvasive precursor to carcinoid tumors and usually presents with obstructive symptoms. We present the case of a 71-year-old female, non-smoker, with a past medical history of asthma, osteoarthritis, allergic rhinitis, and hyperlipidemia who was referred to the pulmonology clinic in view of incidental chest CT findings of multiple pulmonary nodules. Physical examination and labs were unremarkable. CT of the chest showed scattered multiple noncalcified pulmonary nodules with a 10 mm dominant nodule in the inferior right middle lobe and several subcentimeter hypodensities in the left and right lobes of the lung. A PET scan confirmed the CT findings along with no abnormal hypermetabolic activity to suggest malignancy. The patient was followed up in the pulmonology clinic at six months, 12 months, and then 18 months. At 18 months owing to a slight increase in the size of the largest lung nodule, a CT-guided biopsy done was conclusive of a carcinoid. The tumor cells were positive for synaptophysin, chromogranin, insulinoma-associated protein 1 (INSM-1), and thyroid transcription factor 1 (TTF-1). The Ki-67 (Keil) index was <1%. A video-assisted thoracic surgery with right middle lobectomy along with mediastinal lymph node dissection was then done, and the patient was found to have stage pT1aN0 typical carcinoid tumor (1.0 cm), with multiple carcinoid tumors and neuroendocrine hyperplasia, consistent with DIPNECH. She has been under clinical follow-up for over three years at present and continues to be asymptomatic with complete remission following surgery. DIPNECH primarily affects middle-aged, non-smoking females who present with cough and dyspnea, and diagnosis is often delayed due to clinical features overlapping with those of obstructive lung disease. Imaging shows lung nodules, ground-glass opacities, and/or mosaic attenuation. Due to the rarity of the conditions, there are no established clinical trials, and therefore, there is a need to establish guidelines.
摘要：
弥漫性特发性肺神经内分泌细胞增生（DIPNECH）是一种罕见的肺部疾病，其特征是支气管上皮中神经内分泌细胞的弥漫性增殖。它被认为是类癌肿瘤的侵袭前兆，通常表现为阻塞性症状。我们介绍了一个71岁女性的案例，非吸烟者,既往有哮喘病史,骨关节炎,过敏性鼻炎,鉴于多发肺结节的胸部CT表现，高脂血症患者转诊至肺科门诊。体格检查和实验室检查并不明显。胸部CT显示分散的多个非钙化肺结节，右中叶下叶有一个10毫米的优势结节，左叶和右叶有几个亚厘米的低密度。PET扫描证实了CT发现，并且没有异常的高代谢活动提示恶性肿瘤。病人在6个月时在肺科诊所接受随访,12个月,然后是18个月。在18个月时，由于最大的肺结节的大小略有增加，CT引导下的活检证实类癌.肿瘤细胞突触素阳性,嗜铬粒蛋白,胰岛素瘤相关蛋白1（INSM-1），和甲状腺转录因子1(TTF-1)。Ki-67(Keil)指数<1%。然后进行了电视辅助胸腔镜手术，右中肺叶切除术以及纵隔淋巴结清扫术，发现患者患有pT1aN0期典型类癌（1.0cm），多发性类癌肿瘤和神经内分泌增生，与DIPNECH一致。目前，她已经接受了超过三年的临床随访，并且在手术后继续无症状且完全缓解。DIPNECH主要影响中年人，出现咳嗽和呼吸困难的非吸烟女性，由于临床特征与阻塞性肺疾病重叠，诊断通常会延迟。影像学显示肺结节,毛玻璃不透明，和/或马赛克衰减。由于条件的稀缺性，没有确定的临床试验，因此,有必要制定指导方针。
