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弥漫性特发性肺神经内分泌细胞增生症:不寻常的表现。

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Unusual presentation.

作者信息

Alrajhi Nuha Nasser, Paramasivam Muthurajan P, Alboukai Ahmad Amer, Alrikabi Ammar C, Alhamad Esam Hamad

机构信息

Department of Medicine, Division of Pulmonary, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia.

Department of Radiology, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia.

出版信息

Ann Thorac Med. 2019 Apr-Jun;14(2):161-163. doi: 10.4103/atm.ATM_159_18.

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNEC) is an abnormal proliferation of pulmonary neuroendocrine cells that occur without underlying etiology. Here, we report a unique case of 55-year-old female with unusual presentation of DIPNECH and thymoma and on the background history of Crohn's disease that might point toward an autoimmune phenomenon. To the best of our knowledge, there were no previous reports of DIPNECH with either thymoma or Crohn's disease. DIPNECH has premalignant potential and reported in association with carcinoid and non-small cell lung cancer; however, its autoimmune association never reported. The presence of multiple lung nodules along with evidence of small airway disease should alert the physician to include DIPNECH as part of the differential diagnosis given its malignant potential. The prognosis is variable and depends on the presence or absence of underlying malignancy as well as the severity of airflow obstruction.

摘要

弥漫性特发性肺神经内分泌细胞增生症(DIPNEC)是一种无潜在病因的肺神经内分泌细胞异常增殖。在此,我们报告一例独特病例,一名55岁女性,表现出不寻常的DIPNECH合并胸腺瘤,且有克罗恩病病史,这可能提示存在自身免疫现象。据我们所知,此前尚无DIPNECH合并胸腺瘤或克罗恩病的报道。DIPNECH具有恶变潜能,且有与类癌和非小细胞肺癌相关的报道;然而,其与自身免疫的关联从未有过报道。鉴于其恶变潜能,多个肺结节的存在以及小气道疾病的证据应提醒医生将DIPNECH纳入鉴别诊断。预后因人而异,取决于是否存在潜在恶性肿瘤以及气流阻塞的严重程度。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e686/6467018/0d7c0c004d0e/ATM-14-161-g001.jpg

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