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Primitive neuroectodermal tumor of the cervix uteri

Disease definition

Primitive neuroectodermal tumor of the cervix uteri is a rare cancer of cervix uteri derived from neural crest cells, histologically composed of small, round neoplatic cells with variable degree of neural, glial and ependymal differentiation. Macroscopically, the tumor is often a large, soft, poorly circumscribed mass with infiltrative borders and necrotic areas. It presents with dysfuntional vaginal bleeding or discharge, lower abdominal pain and uterine enlargement.

ORPHA:213812

Classification level: Disorder
  • Synonym(s):
    • Cervical malignant peripheral neuroectodermal tumor
    • Cervical peripheral neuroectodermal cancer
    • Malignant peripheral neuroectodermal tumor of the cervix uteri
    • Peripheral neuroectodermal cancer of cervix uteri
  • Prevalence: -
  • Inheritance: -
  • Age of onset: Adult
  • ICD-10: C53.0  C53.1  C53.8
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

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