Hereditary breast and/or ovarian cancer…

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Hereditary breast and/or ovarian cancer syndrome

ORPHA:145Disease

What it is

A genetic condition characterized by hereditary susceptibility to breast and/or ovarian cancer. It can be defined using family history criteria, or through identification of germline pathogenic variants (GPVs) in clinically validated HBOC genes. However, the genetic basis of about half of clinical HBOC is currently unknown or unexplained by single-gene variants, and approximately half of individuals who harbour PVs in HBOC genes do not have a suggestive family history.

Key facts

Age of onset
Adult, Elderly
Inheritance
Autosomal dominant
Classified as
Disease

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

ATMMajor susceptibility factor
BRCA1Disease-causing germline mutation(s) (loss of function)
BRCA2Disease-causing germline mutation(s) (loss of function)
RAD51CDisease-causing germline mutation(s)
RAD51DDisease-causing germline mutation(s)
BARD1Candidate gene tested
BRIP1Candidate gene tested
CHEK2Candidate gene tested
MRE11Candidate gene tested
NBNCandidate gene tested
PALB2Candidate gene tested
PTENCandidate gene tested
RAD50Candidate gene tested
RAD51Candidate gene tested
TP53Candidate gene tested

ICD-10 codes

C50.9ICD-10 uses a narrower term — shared with 1 other rare disease
C56the mapping has not been assessed — shared with 14 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

MESH D061325MONDO 0003582OMIM 114480OMIM 604370OMIM 612555OMIM 613399OMIM 614291UMLS C0677776

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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