Pokhriyal Sindhu C, Sapkota Nisha, Al-Ghuraibawi Muthanna Mohammed Hasan, Pasha Muhammad N, Khan Ahmad Ali, Idris Hadeeqa, Panigrahi Kalpana
Internal Medicine, One Brooklyn Health-Interfaith Medical Center, New York, USA.
Medicine, One Brooklyn Health-Interfaith Medical Center, New York, USA.
Cureus. 2024 Jun 17;16(6):e62527. doi: 10.7759/cureus.62527. eCollection 2024 Jun.
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.
弥漫性特发性肺神经内分泌细胞增生症（DIPNECH）是一种罕见的肺部疾病，其特征为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体，通常表现为阻塞性症状。我们报告一例71岁女性，不吸烟，既往有哮喘、骨关节炎、过敏性鼻炎和高脂血症病史，因胸部CT偶然发现多个肺结节而转诊至肺病诊所。体格检查和实验室检查均无异常。胸部CT显示散在多个非钙化肺结节，右下中叶有一个10 mm的优势结节，左右肺叶有几个亚厘米级的低密度影。PET扫描证实了CT检查结果，且无异常高代谢活动提示恶性肿瘤。患者在肺病诊所分别于6个月、12个月和18个月进行随访。18个月时，由于最大肺结节大小略有增加，进行了CT引导下活检，确诊为类癌。肿瘤细胞突触素、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）呈阳性。Ki-67（基尔）指数<1%。随后进行了电视辅助胸腔镜手术，切除右中叶并清扫纵隔淋巴结，发现患者患有pT1aN0期典型类癌肿瘤（1.0 cm），伴有多个类癌肿瘤和神经内分泌增生，符合DIPNECH。目前她已接受临床随访三年多，术后持续无症状，完全缓解。DIPNECH主要影响出现咳嗽和呼吸困难的中年非吸烟女性，由于临床特征与阻塞性肺病重叠，诊断往往延迟。影像学表现为肺结节、磨玻璃影和/或马赛克样衰减。由于该病罕见，尚无既定的临床试验，因此需要制定指南。
