Primitive neuroectodermal tumor of the…

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Primitive neuroectodermal tumor of the corpus uteri

ORPHA:213630Disease

Also called Malignant peripheral neuroectodermal tumor of the corpus uteri · Peripheral neuroectodermal cancer of the corpus uteri

What it is

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.

Key facts

Classified as
Disease

ICD-10 codes

C54.0ICD-10 uses a narrower term — shared with 5 other rare diseases
C54.1ICD-10 uses a narrower term — shared with 8 other rare diseases
C54.2ICD-10 uses a narrower term — shared with 9 other rare diseases
C54.3ICD-10 uses a narrower term — shared with 5 other rare diseases
C54.8ICD-10 uses a narrower term — shared with 5 other rare diseases

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

MONDO 0016263UMLS C4707724

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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