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mammary adenoid cystic carcinoma

Wednesday 27 April 2016

adenoid cystic carcinoma of the breast

Definition: Adenoid cystic carcinoma of the breast is a rare histological type of triple-negative breast cancer with an indolent clinical behavior, often driven by the MYB-NFIB fusion gene.

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- adenoid cystic carcinoma of the breast

The ductal epithelial cells of ADENOID CYSTIC CARCINOMA are positive for CK and EMA; the myoepithelial cells are positive for SMA, p63 and S100.

Epithelial cells are positive for CD117/ckit, and the myoepithelial cells can be positive as well. Eosinophilic secretions are composed of basement membrane proteins and are positive for PAS with diastase. The lesion is negative for ER, PR and usually negative for Her2.

Unlike the salivary gland, adenoid cystic carcinoma of the breast has a relatively good prognosis.

The lesion should be excised; lymph node dissection is optional, since adenoid cystic carcinoma of the breast does not frequently metastasize.

Adenoid cystic carcinoma of the breast has a favorable prognosis, unlike the salivary gland.

Immunochemistry

The ductal epithelial cells are positive for CD117, CK and EMA; the myoepithelial cells are positive for SMA, p63 and S100, and can be CD117 positive. Eosinophilic secretions are composed of basement membrane proteins and are positive for PAS with diastase. It is occasionally positive for HER2, and negative for ER and PR.

Molecular biology

The MYB-NFIB fusion gene was detected in both adenoid cystic carcinomas and their associated high-grade triple-negative breast cancer components.

Although the distinct components of both cases displayed similar patterns of gene copy number alterations, massively parallel sequencing analysis revealed intratumor genetic heterogeneity.

Adenoid cystic carcinomas of the breast with high-grade transformation are underpinned by the MYB-NFIB fusion gene and, akin to other forms of cancer, may be constituted by a mosaic of cancer cell clones at diagnosis.

The progression from adenoid cystic carcinoma to high-grade triple-negative breast cancer of no special type may involve the selection of neoplastic clones and/or the acquisition of additional genetic alterations.

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- mammary adenoid cystic carcinoma

See also

- adenoid cystic carcinoma

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