# Adenoid cystic carcinoma of the breast

Source: https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/  
OnCo record `adenoid-cystic-carcinoma-breast` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Adenoid cystic carcinoma of the breast is a very rare breast cancer that is triple-negative on testing but behaves almost the opposite of usual triple-negative disease: it seldom reaches the lymph nodes, and nearly everyone is alive at ten years. It is the same tumour type as adenoid cystic carcinoma of the salivary glands and shares its gene fusion.

## Summary

Adenoid cystic carcinoma of the breast is a special type of basal-like carcinoma with a cribriform, tubular or solid pattern of epithelial and myoepithelial cells. In SEER (1977 to 2006) 338 women were diagnosed, an incidence of 0.92 per million person-years, constant over 30 years; 95 percent presented with localised disease, the highest rates were in ER- and PR-negative tumours, age-specific rates rose until midlife and plateaued as for other ER-negative tumours, and relative survival was 98.1 percent at five years, 94.9 percent at ten and 91.4 percent at fifteen, with no excess of other cancers before or after (Ghabach 2010). The t(6;9) translocation fusing MYB to NFIB is a hallmark of adenoid cystic carcinoma of both breast and head and neck, deleting the microRNA target sites that normally repress MYB (Persson 2009). The European Working Group for Breast Screening Pathology lists classical adenoid cystic carcinoma first among the triple-negative special types with indolent behaviour whose patients are unlikely to benefit from chemotherapy, while noting that the solid-basaloid variant behaves more aggressively (Cserni 2021; Thomas 2023). Its 15-year survival above 90 percent is the clearest illustration that triple-negative is a laboratory category, not a prognosis.

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Breast adenoid cystic carcinoma; Breast-ACC; Classic adenoid cystic carcinoma of the breast; Solid-basaloid adenoid cystic carcinoma
- Tags: breast; tnbc; subtype-page
- Group: breast
- Burden: 0.92 cases per million women a year in the United States: 338 women in SEER between 1977 and 2006, 95 percent localised at diagnosis, with rates 39 percent lower in Black than White women (Ghabach 2010).
- Subtypes: Classic adenoid cystic carcinoma (cribriform and tubular; basal-like immunophenotype, indolent); Solid-basaloid adenoid cystic carcinoma (higher grade, more aggressive); Adenoid cystic carcinoma with high-grade transformation
- Biomarkers: Triple-negative receptor status with low grade; MYB-NFIB fusion or MYB overexpression; Myoepithelial markers (p63, CD117) on the basaloid component

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/adenoid-cystic-carcinoma-breast/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/#finding-it [3 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/#treating-it [1 setting]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/#science
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/#living-with-it [7 questions]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/coming/
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/data/ [5 connected records]

## Standard of care

- Localised disease: Surgery with clear margins, radiotherapy after breast conservation, and no chemotherapy for the classical type in the European working group consensus; the solid-basaloid variant is managed as high-grade triple-negative disease. ([Lumpectomy (breast-conserving surgery)](https://onco.cc/terms/lumpectomy/), [Mastectomy](https://onco.cc/terms/mastectomy/), [Sentinel lymph node biopsy](https://onco.cc/technologies/sentinel-node/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Adenoid_cystic_carcinoma
- Ghabach, Breast Cancer Res 2010: adenoid cystic carcinoma of the breast in the United States 1977 to 2006 (SEER): https://doi.org/10.1186/bcr2613
- Persson, PNAS 2009: recurrent MYB-NFIB fusion in adenoid cystic carcinomas of the breast and head and neck: https://doi.org/10.1073/pnas.0909114106
- Cserni, Cancers 2021: triple-negative breast cancer histological subtypes with a favourable prognosis (European Working Group for Breast Screening Pathology): https://doi.org/10.3390/cancers13225694
- Thomas, NPJ Breast Cancer 2023: rare subtypes of triple-negative breast cancer: https://doi.org/10.1038/s41523-023-00554-x

## Connected records

- cancers: [Adenoid cystic carcinoma](https://onco.cc/cancers/adenoid-cystic-carcinoma/), [Invasive cribriform carcinoma of the breast](https://onco.cc/cancers/invasive-cribriform-carcinoma-breast/), [Secretory carcinoma of the breast](https://onco.cc/cancers/secretory-carcinoma-breast/), [Triple-negative breast cancer (TNBC)](https://onco.cc/cancers/tnbc/)
- terms: [Basal-like breast cancer](https://onco.cc/terms/basal-like/), [Grade](https://onco.cc/terms/tumour-grade/), [Lumpectomy (breast-conserving surgery)](https://onco.cc/terms/lumpectomy/), [Mastectomy](https://onco.cc/terms/mastectomy/)
- technologies: [Sentinel lymph node biopsy](https://onco.cc/technologies/sentinel-node/)

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JSON: https://onco.cc/api/v1/entities/adenoid-cystic-carcinoma-breast.json