Breast Pathology

2020 Edition
| Editors: Anna Sapino, Janina Kulka

Adenoid Cystic Carcinoma

  • Maria P. FoschiniEmail author
  • Luca Morandi
Reference work entry


Adenoid cystic carcinoma (AdCC) of the breast is a low-grade tumor, composed of epithelial, myoepithelial, and basal cells, showing features similar to the salivary gland counterpart.

Clinical Features

  • Incidence

    AdCC of the breast is a rare tumor, accounting for less than 1% of all breast tumors.

  • Age

    It usually affects elderly female patients, even if rare cases have been reported in children.

  • Sex

    AdCC usually affects female patients, rare cases have been reported in males.

  • Site

    AdCC is more frequently located in the retroareolar region, but it can affect all the breast quadrants. It can present as a mass of variable size or as screen detected nodule.

  • Treatment

    Radical surgery is the treatment of choice.

  • Outcome

    AdCC is a low malignant potential tumor therefore prognosis is usually good. Recurrences and disease progression are rare. To better predict prognosis, AdCC should be subdivided in three different categories: (i) AdCC classical variant shows good prognosis, with no...

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References and Further Reading

  1. Brill, L. B., Kanner, W. A., Fehr, A., Andrén, Y., Moskaluk, C. A., Löning, T., Stenman, G., & Frierson, H. F., Jr. (2011). Analysis of MYB expression and MYB-NFIB gene fusions in adenoid cystic carcinoma and other salivary neoplasms. Modern Pathology, 24, 1169–1176.PubMedCrossRefPubMedCentralGoogle Scholar
  2. D’Alfonso, T. M., Mosquera, J. M., MacDonald, T. Y., Padilla, J., Liu, Y. F., Rubin, M. A., & Shin, S. J. (2014). MYB-NFIB gene fusion in adenoid cystic carcinoma of the breast with special focus paid to the solid variant with basaloid features. Human Pathology, 45, 2270–2280.PubMedCrossRefPubMedCentralGoogle Scholar
  3. Foschini, M. P., & Krausz, T. (2010). Salivary gland-type tumors of the breast: A spectrum of benign and malignant tumors including “triple negative carcinomas” of low malignant potential. Seminar in Diagnostic Pathology, 27, 77–90.CrossRefGoogle Scholar
  4. Foschini, M. P., Rizzo, A., De Leo, A., Laurino, L., Sironi, M., & Rucco, V. (2016). Solid variant of adenoid cystic carcinoma of the breast: A case series with proposal of a new grading system. International Journal of Surgical Pathology, 24(2), 97–102.Google Scholar
  5. Foschini, M. P., Morandi, L., Asioli, S., Giove, G., Corradini, A. G., & Eusebi, V. (2017). The morphological spectrum of salivary gland type tumours of the breast. Pathology, 49, 215–227.PubMedPubMedCentralCrossRefGoogle Scholar
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  7. Martelotto, L. G., De Filippo, M. R., Ng, C. K., Natrajan, R., Fuhrmann, L., Cyrta, J., Piscuoglio, S., Wen, H. C., Lim, R. S., Shen, R., Schultheis, A. M., Wen, Y. H., Edelweiss, M., Mariani, O., Stenman, G., Chan, T. A., Colombo, P. E., Norton, L., Vincent-Salomon, A., Reis-Filho, J. S., & Weigelt, B. (2015). Genomic landscape of adenoid cystic carcinoma of the breast. Journal of Pathology, 237, 179–189.PubMedCrossRefPubMedCentralGoogle Scholar
  8. Righi, A., Lenzi, M., Morandi, L., Flamminio, F., De Biase, D., Farnedi, A., & Foschini, M. P. (2011). Adenoid cystic carcinoma of the breast associated with invasive duct carcinoma: A case report. International Journal of Surgical Pathology, 19, 230–234.PubMedCrossRefPubMedCentralGoogle Scholar
  9. Shin, S. J., & Rosen, P. P. (2002). Solid variant of mammary adenoid cystic carcinoma with basaloid features: A study of nine cases. American Journal of Surgical Pathology, 26, 413–420.PubMedCrossRefPubMedCentralGoogle Scholar
  10. Vranic, S., Frkovic-Grazio, S., Lamovec, J., Serdarevic, F., Gurjeva, O., Palazzo, J., Bilalovic, N., Lee, L. M., & Gatalica, Z. (2010). Adenoid cystic carcinomas of the breast have low Topo IIα expression but frequently overexpress EGFR protein without EGFR gene amplification. Human Pathology, 41, 1617–1623.PubMedCrossRefPubMedCentralGoogle Scholar
  11. Vranic, S., Gatalica, Z., Deng, H., Frkovic-Grazio, S., Lee, L. M., Gurjeva, O., & Wang, Z. Y. (2011). ER-α36, a novel isoform of ER-α66, is commonly over-expressed in apocrine and adenoid cystic carcinomas of the breast. Journal of Clinical Pathology, 64, 54–57.PubMedCrossRefPubMedCentralGoogle Scholar
  12. Wetterskog, D., Wilkerson, P. M., Rodrigues, D. N., Lambros, M. B., Fritchie, K., Andersson, M. K., Natrajan, R., Gauthier, A., Di Palma, S., Shousha, S., Gatalica, Z., Töpfer, C., Vukovic, V., A’Hern, R., Weigelt, B., Vincent-Salomon, A., Stenman, G., Rubin, B. P., & Reis-Filho, J. S. (2013). Mutation profiling of adenoid cystic carcinomas from multiple anatomical sites identifies mutations in the RAS pathway, but no KIT mutations. Histopathology, 62, 543–550.PubMedPubMedCentralCrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Biomedical and Neuromotor Sciences (DIBINEM), Unit of Anatomic Pathology at Bellaria HospitalUniversity of BolognaBolognaItaly