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胰母细胞瘤

Pancreatoblastoma

ORPHA:677疾病

定义 英文原文(暂无中文)

A rare neoplastic gastroenterologic disease most often found in children, which usually presents with the non-specific symptoms of a palpable mass, vomiting, abdominal pain, jaundice, and weight loss/failure to thrive. Histologically, this malignant epithelial pancreatic neoplasm of the exocrine cells is characterized by multiple lines of differentiation (acinar, ductal, mesenchymal, neuroendocrine) and the presence of squamoid nests.

基本事实

遗传方式
不适用
发病年龄
成年期、儿童期
患病率
<1 / 1 000 000(Europe)

临床表型 9

常见 79–30%7

  • 腹胀 HP:0003270
  • 腹痛 HP:0002027
  • 腹泻 HP:0002014
  • 母体循环甲胎蛋白水平升高 HP:0011432
  • 胰腺钙化 HP:0005213
  • 呕吐 HP:0002013
  • 体重减轻 HP:0001824

偶见 29–5%2

  • 淋巴结形态异常 HP:0002733
  • 黄疸 HP:0000952

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)