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Start free with EleplanPrimitive neuroectodermal tumor of the cervix uteri
ORPHA:213812Disease
Also called Cervical malignant peripheral neuroectodermal tumor · Cervical peripheral neuroectodermal cancer · Malignant peripheral neuroectodermal tumor of the cervix uteri · Peripheral neuroectodermal cancer of cervix uteri
What it is
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.
Key facts
- Prevalence
- <1 / 1 000 000 (annual incidence)
- Age of onset
- Adult
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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