Adenoid Cystic Carcinoma (ACC)

  • Adenoid cystic carcinoma (ACC) of the breast:
    • Is a very rare special histological type of breast cancer:
      • Accounting for approximately 0.1% of all breast tumors
    • It is usually triple negative
    • It is much less likely to have nodal involvement
    • Is more common in postmenopausal women:
      • Most cases are in females
      • The median age of onset is:
        • Between 50 and 60 years
      • With a mean age of 66
    • The typical clinical feature is:
      • A single breast tumor / mass:
        • Multiple nodules are rare
      • Most ACCs are located:
        • Under the areola or in the upper outer quadrants
    • ACC of the breast has no characteristic imaging findings:
      • Ultrasound features are those of:
        • A hypoechoic solid or heterogeneous mass
      • On mammography:
        • The case may present as a lobulated mass with sharp or un sharp margins
      • Nevertheless, these clinical and radiographic features may be similar to any breast cancer:
        • Thus making their precise diagnosis difficult for radiologists
    • Histologically:
      • ACC of the breast typically consists of a dual-cell population of:
        • Luminal and myoepithelial-basal cells:
          • Which are generally negative for estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor receptor 2 (HER2)
      • In addition, some studies have also reported some HR-positive ACC cases:
        • The significance of a positive hormone receptor status is not known:
          • Compared with ACC with negative HR expression, the clinical characteristics and prognosis of this type of ACC are also unknown
    • Distant metastases are rare:
      • However, the lung is the most common site
    • It has a better prognosis than infiltrating ductal triple negative breast cancer:
      • With a 5-year overall survival rate of 88%
      • As prognosis is good:
        • Accurate preoperative diagnosis is important in the determination of suitable treatment
  • References
  • Treitl D, Radkani P, Rizer M, El Hussein S, Paramo JC, Mesko TW. Adenoid cystic carcinoma of the breast, 20 years of experience in a single center with review of literature. Breast Cancer. 2018;25(1)28-33.
  • Welsh JL, Keeney MG, Hoskin TL, et al. Is axillary surgery beneficial for patients with adenoid cystic carcinoma of the breast? J Surg Oncol. 2017;116(6):690-695.
  • Kulkarni N, Pezzi CM, Greif JM, et al. Rare breast cancer: 933 adenoid cystic carcinomas from the National Cancer Data Base. Ann Surg Oncol. 2013;20(7):2236-2241.
  • Kshirsagar AY, Wader JV, Langade YB, Jadhav KP, Zaware SU, Shekhar N. Adenoid cystic carcinoma of the male breast. Int Surg (2006) 91(4):234–6.
  • Pang W, Wang Z, Jin X, Zhang Q. Adenoid cystic carcinoma of the breast in a male: A case report. Med (Baltimore) (2019) 98(32):e16760. doi: 10.1097/MD.0000000000016760
  • Tang W, Peng WJ, Gu YJ, Zhu H, Jiang TT, Li C. Imaging Manifestation of Adenoid Cystic Carcinoma of the Breast. J Comput Assist Tomogr (2015) 39(4):523–30. doi: 10.1097/RCT.
  • Torrao MM, da Costa JM, Ferreira E, da Silva MV, Paiva I, Lopes C. Adenoid cystic carcinoma of the breast. Breast J (2007) 13(2):206.
  • Marchio C, Weigelt B, Reis-Filho JS. Adenoid cystic carcinomas of the breast and salivary glands (or ‘The strange case of Dr Jekyll and Mr Hyde’ of exocrine gland carcinomas). J Clin Pathol (2010) 63(3):220–8. doi: 10.1136/jcp.2009.073908

#Arrangoiz @BreastSurgeon #BreastCancer #AdenocysticCarcinomaoftheBreast #ACC #SurgicalOncologist #CASO #CenterforAdvancedSurgicalOncology #Miami

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