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喉神经内分泌肿瘤

Laryngeal neuroendocrine tumor

ORPHA:100083疾病

定义 英文原文(暂无中文)

A rare head and neck tumor characterized by an epithelial neoplasm with evidence of neuroendocrine differentiation, typically located in the supraglottic larynx. The tumor can be well, moderately, or poorly differentiated, the latter group being subdivided into small cell or large cell neuroendocrine carcinomas. There is a strong association with tobacco use. Patients present with hoarseness, dysphagia, sore throat, airway obstruction, hemoptysis, and rarely a paraneoplastic syndrome due to aberrant hormone production. Poorly differentiated tumors are highly aggressive with high rates of regional and distant metastasis.

基本事实

发病年龄
成年期

临床表型 14

必现 100%2

  • 喉部肿瘤 HP:0100605
  • 神经内分泌肿瘤 HP:0100634

常见 79–30%9

  • 厌食症 HP:0002039
  • 慢性疲劳 HP:0012432
  • 慢性非感染性淋巴结肿大 HP:0002730
  • 发音困难 HP:0001618
  • 循环降钙素浓度升高 HP:0003528
  • 劳力性呼吸困难 HP:0002875
  • 血清5-羟色胺(血清素)水平升高 HP:0003144
  • 口咽部吞咽困难 HP:0200136
  • 体重减轻 HP:0001824

罕见 <4–1%3

  • 促肾上腺皮质激素过多 HP:0011749
  • 循环癌胚抗原水平升高 HP:0031029
  • 抗利尿激素分泌失衡 HP:0031218

外部标识与链接

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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)