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.
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.
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).
Most cancers start in the ducts and drain first to the axillary nodes, which is why the armpit is checked and a sentinel node is sampled.
Same organ: Triple-negative breast cancer (TNBC), Breast cancer (all types), HR-positive / HER2-negative breast cancer, HER2-positive breast cancer, Male breast cancer, Ductal carcinoma in situ (DCIS), High-risk early HR-positive breast cancer, HR-positive metastatic breast cancer after CDK4/6 inhibitors, HER2-low and HER2-ultralow metastatic breast cancer, Early HER2-positive breast cancer, HER2-positive breast cancer with brain metastases, Early triple-negative breast cancer, Metastatic triple-negative breast cancer, Basal-like 1 triple-negative breast cancer (BL1), Basal-like 2 triple-negative breast cancer (BL2), Mesenchymal triple-negative breast cancer (M), Mesenchymal stem-like triple-negative breast cancer (MSL), Luminal androgen receptor triple-negative breast cancer (LAR), Immunomodulatory triple-negative breast cancer (IM), Metaplastic breast carcinoma, Carcinoma with medullary pattern (medullary breast cancer), Apocrine carcinoma of the breast, Secretory carcinoma of the breast, BRCA-associated triple-negative breast cancer, Inflammatory breast cancer, Paget disease of the nipple, Phyllodes tumour of the breast, Invasive lobular carcinoma of the breast, Invasive breast carcinoma of no special type (invasive ductal carcinoma), Tubular carcinoma of the breast, Mucinous carcinoma of the breast, Papillary carcinomas of the breast (encapsulated, solid and invasive papillary), Invasive cribriform carcinoma of the breast, Invasive breast carcinoma with medullary pattern (medullary carcinoma), Invasive micropapillary carcinoma of the breast, Neuroendocrine neoplasms of the breast, Lobular carcinoma in situ (LCIS)
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.
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Query for this cancer: (TITLE:"Adenoid cystic carcinoma of the breast" OR ABSTRACT:"Adenoid cystic carcinoma of the breast" OR TITLE:"Breast adenoid cystic carcinoma" OR ABSTRACT:"Breast adenoid cystic carcinoma" OR TITLE:"Breast-ACC" OR ABSTRACT:"Breast-ACC" OR TITLE:"Classic adenoid cystic carcinoma of the breast" OR ABSTRACT:"Classic adenoid cystic carcinoma of the breast" OR TITLE:"Solid-basaloid adenoid cystic carcinoma" OR ABSTRACT:"Solid-basaloid adenoid cystic carcinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Adenoid cystic carcinoma of the breast, not a curated reading list.
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