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Adenoid Cystic Carcinoma

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  • First Online:
Breast Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

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Definition

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

  • 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.

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  • 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.

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  • 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.

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  • 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.

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  • 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.

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  • Fusco, N., Geyer, F. C., De Filippo, M. R., Martelotto, L. G., Ng, C. K., Piscuoglio, S., Guerini-Rocco, E., Schultheis, A. M., Fuhrmann, L., Wang, L., Jungbluth, A. A., Burke, K. A., Lim, R. S., Vincent-Salomon, A., Bamba, M., Moritani, S., Badve, S. S., Ichihara, S., Ellis, I. O., Reis-Filho, J. S., & Weigelt, B. (2016). Genetic events in the progression of adenoid cystic carcinoma of the breast to high-grade triple-negative breast cancer. Modern Pathology, 29, 1292–1305.

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  • 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.

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  • 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.

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  • 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.

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  • 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.

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  • 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.

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Correspondence to Maria P. Foschini .

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Foschini, M.P., Morandi, L. (2020). Adenoid Cystic Carcinoma. In: Sapino, A., Kulka, J. (eds) Breast Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-62539-3_4697

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  • DOI: https://doi.org/10.1007/978-3-319-62539-3_4697

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-62538-6

  • Online ISBN: 978-3-319-62539-3

  • eBook Packages: MedicineReference Module Medicine

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