Low-grade neuroendocrine tumor of the…

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Low-grade neuroendocrine tumor of the corpus uteri

ORPHA:213736Disease

Also called Low-grade neuroendocrine tumor of the uterine corpus · Well-differentiated neuroendocrine neoplasm of the endometrium · Well-differentiated neuroendocrine tumor of the corpus uteri · Well-differentiated neuroendocrine tumor of the endometrium

What it is

Low-grade neuroendocrine tumor of the corpus uteri is an extremely rare uterine cancer typically characterized by a well demarcated, solid, frequently pedunculated tumor originating from neuroendocrine cells scattered within the endometrium, often associated with ectopic hormone production. Patients usually present with vaginal bleeding or discharge and a pelvic mass with a polypoid tumor sometimes protruding through the cervical canal. Symptoms related to ectopic hormone production (flushing, sweating, diarrhea, bronchospasm) may also develop.

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 0016270UMLS C5190779

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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