支气管神经内分泌肿瘤
Bronchial neuroendocrine tumor
定义 英文原文（暂无中文）
A rare neuroendocrine neoplasm characterized by origin from pulmonary neuroendocrine cells and ranging from low-grade typical carcinoid and intermediate-grade atypical carcinoid to high-grade large-cell neuroendocrine carcinoma and small-cell carcinoma. Two thirds of the tumors are located in the major bronchi, with a predilection for the right lung, in particular the middle lobe. Most patients with central bronchial tumors present with hemoptysis, cough, recurrent pulmonary infections, fever, chest discomfort, and unilateral wheezing, while peripheral carcinoids are usually discovered only incidentally. Carcinoid syndrome or Cushing syndrome are very rare. The tumors may be part of multiple endocrine neoplasia type 1.
别名
支气管NET
基本事实
- 遗传方式
- 不适用
- 发病年龄
- 青少年期、成年期、儿童期、老年期
临床表型 31
必现 100%1
- 肺类癌 HP:0030445
常见 79–30%12
- 肺间质形态异常 HP:0006530
- 厌食症 HP:0002039
- 哮喘 HP:0002099
- 胸痛 HP:0100749
- 慢性非感染性淋巴结肿大 HP:0002730
- 呼吸困难 HP:0002094
- 咯血 HP:0002105
- 干咳 HP:0031246
- 肺炎 HP:0002090
- 食欲不振 HP:0004396
- 体重减轻 HP:0001824
- 哮鸣音 HP:0030828
罕见 <4–1%18
- 肺动脉瓣尖瓣形态异常 HP:0031566
- 排便紧迫感 HP:0012701
- 支气管收缩 HP:4000007
- 心源性休克 HP:0030149
- 系统性疾病的皮肤表现 HP:0001005
- 生长激素水平升高 HP:0000845
- 面部毛细血管扩张 HP:0007380
- 肝功能衰竭 HP:0001399
- 肝脏肿大 HP:0002240
- 低血压 HP:0002615
- 血促肾上腺皮质激素（ACTH）水平升高 HP:0003154
- 血皮质醇水平增加 HP:0003118
- 血清5-羟色胺（血清素）水平升高 HP:0003144
- 盗汗 HP:0030166
- 心悸 HP:0001962
- 迁延性腹泻 HP:0004385
- 右心衰竭 HP:0001708
- 三尖瓣反流 HP:0005180
外部标识与链接
发现这一页有错误？告诉我 · 邮件主题会自动带上本页的 ORPHA 编号
本页数据来源
- 疾病定义、同义词、基因、表型、流行病学：Orphanet（CC BY 4.0）
- 中文病名：Orphanet 中文包，冻结于 2020-06-01
- 表型中文标签：HPO 简体中文翻译（CHPO 上游成果）