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Primitive neuroectodermal tumor of the corpus uteri

Disease definition

Primitive neuroectodermal tumor of the corpus uteri is a rare cancer of corpus uteri derived from neural crest cells, characterized by small, round neoplastic cells with variable degree of neural, glial and ependymal differentiation. Macroscopically, the tumor is often a large, poorly circumscribed polypoid mass with necrotic areas and hemorrhage. It usually presents with lower abdominal or pelvic pain, irregular vaginal bleeding or discharge, pelvic mass and uterine enlargement.

ORPHA:213630

Classification level: Disorder
  • Synonym(s):
    • Malignant peripheral neuroectodermal tumor of the corpus uteri
    • Peripheral neuroectodermal cancer of the corpus uteri
  • Prevalence: -
  • Inheritance: -
  • Age of onset: -
  • ICD-10: C54.0  C54.1  C54.2  C54.3  C54.8
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

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