Stefański Mariusz, Bruliński Krzysztof, Stefańska Marianna
Oddział Chirurgii Klatki Piersiowej, Centrum Pulmonologii I Torakochirurgii, Bystra, Polska.
Oddział Chorob Wewnętrznych, Centrum Pulmonologii I Torakochirurgii, Bystra, Polska.
Wiad Lek. 2017;70(5):1005-1012.
Pulmonary neuroendocrine cells (PNEC) are present in the normal lungs with the incidence of 1 in 2500 epithelial cells. They usually proliferate in the presence of reactive processes related to inflammation and fibrosis of the lung parenchyma. The division of pulmonary neuroendocrine cell hyperplasia proposed by Travis et al. additionally distinguished diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) or proliferation that occurs in people without reactive hyperplasia risk factors. The confirmation of the DIPNECH diagnosis requires staining of biopsy specimens using the immunohistochemical technique for neuroendocrine markers.
The aim of this study is to overview the cases of 5 patients in whom the histopathological DIPNECH diagnosis was made in the process of invasive diagnostics performed at the Department of Thoracic Surgery. The aim of the study is to evaluate typical clinical, functional, radiological and histopathological features of this rare disease syndrome.
In the period from April 2010 to June 2014, five patients with lesions in the lungs were subjected to invasive diagnostics. Histopathological and immunohistochemical examinations of the collected specimens were used to make the DIPNECH diagnosis in these patients. The natural history of the disease was traced based on a 5-year follow-up in one of the patients. In addition, we analyzed the literature with regard to the described cases.
Thanks to the early diagnosis of non-specific lesions in the lungs, typical carcinoid which develops on the basis of discussed DIPNECH, was found in the resected material in two out of five operated patients. The accurate diagnosis of DIPNECH allows for the implementation of appropriate treatment and channels further management of the patient into the right direction.
肺神经内分泌细胞(PNEC)存在于正常肺组织中,其发生率为每2500个上皮细胞中有1个。它们通常在与肺实质炎症和纤维化相关的反应性过程中增殖。Travis等人提出的肺神经内分泌细胞增生分类,进一步区分了弥漫性特发性肺神经内分泌细胞增生(DIPNECH)或在无反应性增生风险因素的人群中发生的增殖。DIPNECH诊断的确认需要使用神经内分泌标志物的免疫组织化学技术对活检标本进行染色。
本研究的目的是概述在胸外科进行的侵入性诊断过程中,经组织病理学诊断为DIPNECH的5例患者的病例情况。本研究的目的是评估这种罕见疾病综合征的典型临床、功能、放射学和组织病理学特征。
在2010年4月至2014年6月期间,对五例肺部有病变的患者进行了侵入性诊断。通过对收集标本进行组织病理学和免疫组织化学检查,对这些患者做出DIPNECH诊断。对其中一名患者进行了5年随访,以追踪疾病的自然史。此外,我们还分析了关于所描述病例的文献。
由于对肺部非特异性病变的早期诊断,在五例接受手术的患者中,有两例在切除的材料中发现了基于所讨论的DIPNECH发展而来的典型类癌。DIPNECH的准确诊断有助于实施适当的治疗,并将患者的进一步管理引导到正确的方向。