Cassar Peter, Buhagiar Dione, Gauci Jonathan, Bartolo Kyra, Mizzi Adrian, Mallia Andrew, Micallef Josef
Department of Nephrology, Mater Dei Hospital, Msida, Malta.
Department of Internal Medicine, Mater Dei Hospital, Msida, Malta.
Eur J Case Rep Intern Med. 2024 Sep 23;11(10):004870. doi: 10.12890/2024_004870. eCollection 2024.
A middle-aged woman undergoing a computed tomography scan while being investigated for a retrosternal goitre, was found to have several solid intrapulmonary nodules of varying sizes with mosaic attenuation of lung parenchyma. After serial radiology follow-up, a radiologist with a special interest in thoracic imaging made the tentative diagnosis of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) during discussions at the local multidisciplinary team meeting. Radionuclide imaging was performed to assist in reaching a diagnosis. Uptake of DOTATATE by the pulmonary nodules on a background of mosaic attenuation pattern supported a diagnosis of DIPNECH. Potential secondary metastatic disease from previous breast malignancy confounded a possible earlier diagnosis of DIPNECH, with subsequent diagnostic imaging modalities leading to the rare diagnosis. The patient was treated symptomatically with oral steroids with no improvement, and subsequently with octreotide which significantly improved her condition.
Clinical symptoms of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) are common to several other respiratory conditions that are found ubiquitously in the community; being aware of this rare condition will help in reaching a diagnosis in a timelier manner.Several potential treatments are described in the literature including steroid therapy, cytotoxic agents and somatostatin analogues, which despite their efficacy have not been demonstrated in studies; however, a small number of case reports such as this one showed an improvement in symptomatology with this treatment.
一名中年女性因胸骨后甲状腺肿接受计算机断层扫描检查时,发现肺部有多个大小不一的实性结节,肺实质呈马赛克样衰减。经过一系列影像学随访后,一位对胸部影像有特殊兴趣的放射科医生在当地多学科团队会议讨论期间初步诊断为弥漫性特发性肺神经内分泌细胞增生症(DIPNECH)。进行了放射性核素成像以协助诊断。在马赛克样衰减背景下,肺部结节对DOTATATE的摄取支持了DIPNECH的诊断。既往乳腺恶性肿瘤可能的继发性转移疾病干扰了DIPNECH的早期诊断,随后的诊断性成像方法导致了这一罕见诊断。患者接受口服类固醇对症治疗但无改善,随后使用奥曲肽治疗,病情显著改善。
弥漫性特发性肺神经内分泌细胞增生症(DIPNECH)的临床症状与社区中普遍存在的其他几种呼吸系统疾病相同;了解这种罕见疾病将有助于更及时地做出诊断。文献中描述了几种潜在的治疗方法,包括类固醇疗法、细胞毒性药物和生长抑素类似物,尽管它们的疗效尚未在研究中得到证实;然而,像这样的少数病例报告显示这种治疗方法能改善症状。