Understanding Adenoid Cystic Breast Cancer

Diagnosis and Prognosis of Adenoid Cystic Breast Cancer

Microscopic Image of Breast Carcinoma
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If you've been diagnosed with adenoid cystic breast cancer you're likely feeling anxious, especially as much less information is available on this uncommon type of breast cancer. Fortunately, the prognosis of this type of cancer is excellent. Let's look at how this unique type of breast cancer is usually diagnosed and treated.

Description of Adenoid Cystic Breast Carcinoma

The microscopic cell that adenoid cystic cancers originate from is still unknown.

But under the microscope, these cancer cells resemble those of an adenoid cystic carcinoma of the salivary gland.

Usually not aggressive, this type of breast cancer has a good chance of recovery after treatment. Adenoid cystic carcinoma (ACC) is not a breast fibroadenoma nor is it a breast cyst, both of which are benign breast conditions.

Not all cases of ACC are fueled by estrogen or progesterone, but some may be hormone-sensitive. 

Adenoid cystic breast carcinoma may also be referred to as adenoid cystic breast cancer, ACC, ACCB, AbCC, adenocystic carcinoma, or cylindroma.

Incidence of Adenoid Cystic Carcinoma

ACC of the breast is uncommon, accounting for less than 0.1% of all breast cancers, with the majority of people being postmenopausal women between the ages of 50 and 60. ACC has also been found in men.

Signs and Symptoms of ACC

ACC usually is noted after a subareolar lump (lump under the area surrounding the nipple) or breast pain is noted.

When a lump is present it can vary in size from very small to very large.  

Tests Used to Diagnose Adenoid Cystic Carcinoma

A number of different studies may be done to diagnose the condition including:

  • Mammogram. ACC does not have a lot of distinct findings on mammogram and an abnormality may look either malignant or benign. The size of an abnormality seen on mammogram may also vary significantly from the size of the lesion found at surgery.
  • Breast ultrasound. An ACC tumor may be bumpy or have a fuzzy outline and an irregular shape on an ultrasound.
  • Breast biopsy. Either a fine needle aspiration biopsy (preferred), 
  • AC
  • core needle biopsy or open surgical biopsy is usually used to take a sample of tissue to study under the microscope. 
  • Sentinel lymph node biopsy. A sentinel lymph node biopsy is usually done at the time of surgery to check for any cancer cells which have spread to the lymph nodes. 

Treatments for Adenoid Cystic Carcinoma

Treatment options for adenoid cystic carcinoma are similar to other breast cancers, though fewer of these tumors are estrogen receptor positive and therefore hormonal treatments are used less often. While surgery is the preferred first-line treatment, it's not known what adjunctive treatment methods are best since the condition is so uncommon. Options include:

  • Lumpectomy, a surgery to remove the tumor and a wide margin of surrounding tissue.
  • Mastectomy, a surgery to remove all of the breast tissue, for larger tumors.
  • Radiation may be done after a lumpectomy to help prevent recurrence, especially with larger tumors.
  • Hormone therapy for hormone-sensitive tumors.
  • HER2 targeted therapy for HER2 positive tumors (rare).

    Prognosis of Adenoid Cystic Carcinoma

    The prognosis of ACC is usually very good. Most of these cancers do not spread to the lymph nodes, and they rarely metastasize to other parts of the body. When they do spread, the most common site is the lung.

    Unlike many breast cancers, the risk of recurrence after effective treatment is very low. Overall, these tumors have an excellent outlook for complete recovery, though long-term follow-up is still important.

    A Word From Verywell on Adenoid Cystic Carcinoma of the Breast

    ACC is an uncommon form of breast cancer found most commonly in women between the ages of 50 and 60.

    Despite often being "triple negative," meaning that it is usually estrogen receptor and HER2 receptor negative, it tends to have a very good prognosis. Surgery is the mainstay of treatment with the role of chemotherapy and radiation therapy currently unknown. While it may sometimes metastasize (to areas such as the lungs, kidneys, and thyroid gland), the chance of recurrence is low relative to other types of cancer.

    While ACC has a good prognosis, having any type of breast cancer of any prognosis can be terrifying. It's important to learn what you can about your cancer. Since this tumor is uncommon, you may want to receive care at one of the larger NCI designated cancer centers which have oncologists more likely to be familiar with uncommon cancers.

    Sources:

    Canyilmaz, E., Uslu, G., Memis, Y. et al. Adenoid Cystic Carcinoma of the Breast: A Case Report and Literature Review. Oncology Letters. 2014. 7(5):1599-1601.

    Yan, Z., Leong, M., and G. Lim. Discordant Correlation of Breast Adenoid Cystic Carcinoma on Imaging and Pathology: A Case Report and Literature Review on Surgical Management. International Journal of Surgical Case Reports. 2017. 42:196-199.

    Yang, H. Primary Male Breast Adenoid Cystic Carcinoma—A Case Report and Review of the Literature. Clinical Studies and Medical Case Reports. 2(4):1-3.