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Isolated melanotic schwannoma

ORPHA:590539疾病暂无中文名

定义 英文原文(暂无中文)

A rare nervous system tumor characterized by cells phenotypically representing Schwann cells but containing melanosomes and expressing melanoma markers. The entity occurs as a non-psammomatous (typically affecting spinal nerves) or a psammomatous (also involving nerves of the intestinal tract and heart) variant. About 50% of psammomatous tumors are associated with Carney complex. Slightly over 10% of melanotic schwannomas follow a malignant course.

别名

Isolated melanocytic schwannoma

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

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;本条目在该版本中无中文名
  • 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)