Name l Define l Risk l Detect l Treatment l Stage/Grade l Followup l Mets/Recur l Link
Names - Synonyms
The following names have referred to adenoid cystic carcinoma of the breast, either correctly or erroneously: ACC-M/B (metastatic adenoid cystic carcinoma - breast; AdCC - breast, ACC - breast; adenocystic basaloid carcinoma - breast; adenocystic basal cell carcinoma - breast; adenocystic carcinoma - breast; adenoid cystic carcinoma - breast, adenomyoepithelioma - breast; basaloma - breast; carcinoma adenoides cysticum - breast; cribriform; cylindroid adenocarcinoma - breast; cylindromatous breast; cylindroma - breast; malignant cylindroma - breast; pseudoadenomatous basal cell carcinoma - breast;
Adenoid Cystic Carcinoma of the breast is a rare, well differentiated, and usually slow growing malignant tumor, accounting for less than 0.1% of all breast cancers. It commonly starts in the glandular tissue of the breast as a firm, sometimes tender or painful, movable mass. This cancer generally has a better prognosis than most forms of breast cancer, although distant metastases and death have occurred.
Risk Factors - Causes
Age is of no consequence to the diagnosis of this cancer. Literature has reported cases between 19 and 90 years of age in both females and males. As of this writing, there were no commonly accepted risk factors reported in the literature.
Patients may present with a slow growing, solid feeling, movable mass for months, even years, prior to actual diagnosis. A common symptom of this cancer is intermittent pain and tenderness in the breast mass. It also tends to develop in the area of the nipple or areola. On mammography, these tumors often appear as small lobulated nodules with clearly defined borders, but they may also show as larger mass with more ill-defined margins. Because of the rarity of this cancer, it is not easily recognizable by pathologists. A second , independent, pathology assessment is recommended.
The most commonly accepted primary treatment for this cancer is complete surgical excision with clean margins. Mastectomy, partial mastectomy, and lumpectomy have all been used to accomplish this. The statistical numbers for each have been too small to clearly confirm which of these surgical treatments may be best. Radiation therapy has been used but it's effectiveness has not been proven. Chemotherapy is unproven, also. Since the large majority of cases are estrogen receptor negative, anti estrogen therapy is not beneficial.
Stage - Grade
The following grading system has been defined in literature:
grade I: completely glandular
grade II: < 30% solid areas
grade III: > or = 30% solid pattern
Earlier studies concluded that a solid variant of adenoid cystic carcinoma of the breast had a more aggressive clinical course, but other reports did not confirm this. Perineural invasion is possible. Axillary lymph node involvement is extremely rare.
Follow ups should include imaging of the original site of the tumor and lung imaging. Imaging of the liver, bones, brain, and other organs may be ordered if symptoms are present. Since recurrence and metastasis have been reported at 20+ years from original diagnosis, these follow ups should be done for the rest of the patient's life.
Metastasis - Recurrence
Recurrence after complete surgical excision is uncommon, although it has been reported in literature. This may have been caused by unclean or questionable surgical margins. Metastasis has been reported and personally experienced by the author of this document. The most common body site for metastasis is the lung. Other metastatic body sites reported include the liver, bones, lymph nodes, soft tissue, brain, and kidney. This generally occurs without lymph node involvement. In fact, lymph node involvement is extremely rare with this cancer.
Local recurrence can safely be treated with one of several wide excisional surgical procedures. Metastasis have been treated by excisional surgery, radiotherapy, chemotherapy, and stereotactic radiation therapy. Since statistics for metastatic adenoid cystic carcinoma of the breast are minute, none of these treatments have been conclusively proven.
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