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ACC of the breast has been reported in few cases only. Carriers of inherited mutations in the<jats:italic>BRCA1<\/jats:italic>gene are prone to the development of breast cancer, mainly invasive ductal or medullary type carcinomas. We describe for the first time a<jats:italic>BRCA1<\/jats:italic>mutation carrier with a diagnosis of ACC of the breast.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>The patient developed an invasive ductal carcinoma (IDC) at the age of 40 years and an ACC in the contralateral breast at 44 years. Immunohistochemical examination of the ACC revealed a triple negative status (i.e., negativity for estrogen receptor, progesterone receptor and HER2 protein) and positivity for p53. Using a combination of loss of heterozygosity (LOH) and sequencing analyses, the loss of the wild-type<jats:italic>BRCA1<\/jats:italic>allele was detected in both the ACC and the IDC. In addition, two different somatic<jats:italic>TP53<\/jats:italic>mutations, one in the ACC only and another one in the IDC only, were observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Both the immunohistochemical and molecular features observed in the ACC are typical of<jats:italic>BRCA1<\/jats:italic>-associated breast cancers and suggest an involvement of the patient\u2019s germline mutation in the disease. The occurrence of rare histological types of breast cancers, including malignant phyllodes tumor, atypical medullary carcinoma and metaplastic carcinoma, in<jats:italic>BRCA1<\/jats:italic>mutation carriers has been already reported. Our findings further broaden the spectrum of<jats:italic>BRCA1<\/jats:italic>-associated breast malignancies.<\/jats:p><\/jats:sec>","DOI":"10.1186\/1471-2407-13-46","type":"journal-article","created":{"date-parts":[[2013,2,2]],"date-time":"2013-02-02T00:34:08Z","timestamp":1359765248000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["First description of an acinic cell carcinoma of the breast in a BRCA1 mutation carrier: a case report"],"prefix":"10.1186","volume":"13","author":[{"given":"Carla B","family":"Ripamonti","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mara","family":"Colombo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrizia","family":"Mondini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manoukian","family":"Siranoush","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernard","family":"Peissel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Loris","family":"Bernard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Radice","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Luisa","family":"Carcangiu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2013,2,1]]},"reference":[{"key":"3816_CR1","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1002\/(SICI)1097-0347(199907)21:4<297::AID-HED2>3.0.CO;2-R","volume":"21","author":"HT Hoffman","year":"1999","unstructured":"Hoffman HT, Karnell LH, Robinson RA, Pinkston JA, Menck HR: National cancer data base report on cancer of the head and neck: acinic cell carcinoma. 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