What are the other Names for this Condition? (Also known as/Synonyms)

• Mammary Malignant Adenomyoepithelioma
• MAME of Breast

What is Malignant Adenomyoepithelioma of Breast? (Definition/Background Information)

• Breast cancer is the most common type of cancer diagnosed in women. It is a type of cancer in which certain cells in the breast become abnormal, grow uncontrollably, and form a malignant mass (tumor). There are various types of breast cancers
• Malignant Adenomyoepithelioma of Breast is a rare form of breast cancer. Adenomyoepithelioma is a benign tumor that consists of myoepithelial cells and epithelial cells. When there is a malignant transformation occurring within adenomyoepithelioma, it is called malignant adenomyoepithelioma (MAME). However, such transformations are very rare occurrences
• The cause of formation of Malignant Adenomyoepithelioma of Breast is not well-established. The condition causes a painless breast lump that grows in size. The malignancy may be diagnosed via a breast biopsy
• The treatment of Malignant Adenomyoepithelioma of Breast usually involves complete removal of the tumor i.e., “true surgical resection”. Depending on the stage of the malignancy, radiotherapy and/or chemotherapy may be necessary
• The prognosis depends on many factors, but in particular, the stage of the tumor. Since the risk for recurrence and metastasis (spread of tumor to local and regional areas) is high, close clinical monitoring is necessary with periodic follow-ups. An early diagnosis and adequate treatment can significantly improve the outcomes

Who gets Malignant Adenomyoepithelioma of Breast? (Age and Sex Distribution)

• Malignant Adenomyoepithelioma of Breast is seen in young, middle-aged, and older adults. The age of onset at the time of diagnosis is between 26 to 81 years
• Even though both males and females are affected, a higher number of cases are reported in women than men
• All racial and ethnic groups are affected, and no specific predilection is seen

What are the Risk Factors for Malignant Adenomyoepithelioma of Breast? (Predisposing Factors)

The risk factors for Malignant Adenomyoepithelioma of Breast are not well established since the condition is very rare.

In general, the risk factors for developing breast cancer may include:

• Women have a much greater chance of developing breast cancer than men
• Age: The risk increases for women over the age of 55 years
• Personal history: Having cancer in one breast, puts a woman at risk for having cancer in the other breast as well
• Family history: Women with a mother, sister, or daughter diagnosed with breast cancer have a higher risk for developing the condition
• Inherited gene mutations: Mutations in certain genes (BRCA1 or BRCA2) can lead to a much higher risk
• Radiation therapy: Receiving radiation therapy to the chest or breast area can also increase the risk. It is noted that the risk for developing breast cancer later in life is the greatest for radiation therapy administered during puberty
• Obesity: Being overweight or obese elevates the risk after menopause
• Alcohol use: Consuming alcohol more than one drink per day; the more the alcohol consumed, the greater is the risk
• Menstrual cycle: Women who got their period before the age of 12 years, and those who reached menopause after age 55 have an increased risk
• Postmenopausal hormone therapy: Women taking hormone replacement therapy medications containing both estrogen and progesterone for menopause have a higher risk of developing breast cancer
• Reproductive history: Having the first child after the age of 35, or never having children
• Physical inactivity: A lack of physical exercise (leading a sedentary life) can increase the risk
• Breastfeeding: Not breastfeeding one's child can increase the risk for developing the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Malignant Adenomyoepithelioma of Breast? (Etiology)

• The exact cause of development of Malignant Adenomyoepithelioma of Breast is currently not well understood
• Some believe that the malignant condition occurs from a previous benign or low-grade adenomyoepithelioma

What are the Signs and Symptoms of Malignant Adenomyoepithelioma of Breast?

The signs and symptoms of Malignant Adenomyoepithelioma of Breast may include:

• A growing painless lump or mass
• Presence of a firm mass in the breast that may measure up to 7 cm in size

In rare cases, the signs and symptoms may include the following which can be seen with other breast cancer types:

• Bloody discharge from the nipple
• Thickening or swelling of part of the breast; change in the size or shape of the breast
• Inversion of the nipple (pulling-in of nipple into the breast) 
• Changes to the skin covering the breast or nipple area, including dimpling, irritation, redness, scaling, peeling, or puckering 
• The condition can be bilateral affecting both the breasts

How is Malignant Adenomyoepithelioma of Breast Diagnosed?

Malignant Adenomyoepithelioma of Breast may be diagnosed in the following manner:

• Complete physical examination with comprehensive medical and family history evaluation
• Breast exam to check for any lumps or unusual signs in the breasts
• Blood tests including complete blood count (CBC) test
• Mammogram: A mammogram uses X-rays to provide images of the breast. On a mammogram, the tumors are usually well defined with calcifications
• Galactography: A mammography using a contrast solution, mostly used to analyze the reason behind a nipple discharge
• Breast ultrasound scan: Using high-frequency sound waves to produce images of the breast. Ultrasound studies may show a well-defined nodule that may have solid and/or cystic areas
• Computerized tomography (CT) or magnetic resonance imaging (MRI) scan of the breast
• Positron emission tomography (PET) scan to help determine, if the cancer has spread to other organ systems
• Breast biopsy:
  • A biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform additional studies, which may include immunohistochemical stains and molecular studies to assist in the diagnosis

Biopsies are the only methods used to determine whether an abnormality is benign or cancerous. These are performed by inserting a needle into a breast mass and removing cells or tissues, for further examination. There are different types of biopsies:

• Fine needle aspiration biopsy (FNAB) of breast mass: In this method, a very thin needle is used to remove a small amount of tissue
• Core needle biopsy of breast mass: A wider needle is used to withdraw a small cylinder of tissue from an abnormal area of the breast
• Open tissue biopsy of breast mass: A surgical procedure used less often than needle biopsies, it is used to remove a part or all of a breast lump for analysis

A malignancy is diagnosed when tumor samples are examined under a microscope. The pathologist diagnoses the malignant condition depending on the pattern of growth, cellular atypia, and mitotic activity within the tumor. When examined under the microscope, either the ductal epithelial cells or the myoepithelial cells may be malignant. In some cases, both the ductal epithelial cells and myoepithelial cells or malignant. Additional immunohistochemical stains or special studies may be necessary to diagnose the condition.

The differential diagnosis to exclude conditions with similar presentations include:

• Adenoid cystic carcinoma of breast
• Adenoma of breast
• Fibroadenoma of breast
• Intraductal papilloma of breast with mild epithelial hyperplasia
• Low-grade adenosquamous carcinoma of breast
• Metaplastic carcinoma of breast
• Papillary carcinoma of breast
• Sclerosing adenosis of breast
• Tubular carcinoma of breast

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Malignant Adenomyoepithelioma of Breast?

The complications of Malignant Adenomyoepithelioma of Breast may include:

• Emotional distress due to the presence of breast cancer
• Recurrences following surgery is commonly observed; incompletely excised tumors are known to generally recur
• Metastasis of the tumor to local and regional sites; the tumor is known to metastasize to the lymph nodes, liver, lung, brain, bone, thyroid, skin and kidney
• Side effects of chemotherapy, which may include nausea, vomiting, hair loss, decreased appetite, mouth sores, fatigue, low blood cell counts, and a higher chance of developing infections
• Side effects of radiation therapy that may include sunburn-like rashes, where radiation was targeted, red or dry skin, heaviness of the breasts, and general fatigue
• Lymphedema (swelling of an arm) may occur after surgery or radiation therapy, due to restriction of flow of lymph fluid resulting in a build-up of lymph. It may form weeks to years after treatment that involves radiation therapy to the axillary lymph nodes

How is Malignant Adenomyoepithelioma of Breast Treated?

Treatment options available for individuals with Malignant Adenomyoepithelioma of Breast are dependent upon the following:

• Type of cancer
• The staging of the cancer: If a breast cancer is diagnosed, staging helps determine whether it has spread, and what treatment options are best-suited for the patient
• Whether the cancer cells are sensitive to certain particular hormones, and
• One’s personal preferences

Nevertheless, the optimal treatment for the condition is still controversial since Malignant Adenomyoepithelioma of Breast is a very rare malignancy.

Surgery: Surgery is generally the most common form of treatment involving the removal of the tumor. Various types of surgery to remove the malignant tumor include:

• Lumpectomy: Breast-sparing surgery (least invasive breast cancer surgery) in which the tumor, as well as a small portion of the surrounding tissue is removed
• Mastectomy: Surgery to remove all of the breast tissue; it may be simple (removal of the breast, nipple, areola, sentinel lymph nodes) or radical mastectomy (removal of the breast, nipple, areola, all axillary lymph nodes, and underlying muscle of the chest wall)
• Sentinel node biopsy: Procedure done to examine the “sentinel lymph node,” or lymph node(s) closest to the tumor, as this is the most likely location, where cancer cells may have spread to. This lymph node is removed and tested for cancerous cells
• Axillary node dissection: This procedure is performed to remove some axillary lymph nodes in the underarm area, to allow dissection and examination. This helps in establishing whether the cancer has spread to more than one lymph node

Other treatment options may include chemotherapy and radiation therapy.

• Radiotherapy can be used as primary therapy in situations where the tumor cannot be removed completely, or when the tumor reappears after surgery
• Radiotherapy can also be used as additional therapy after surgery, if there is a possibility of tumor recurrence after surgery, or if there are inadequate margins (possibility of tumor left behind) following surgery. In some cases, due to location of tumor, a complete surgical removal of the tumor is difficult
• Chemotherapy can be used for treating the tumor in the following conditions:
  • When the tumors cannot be removed completely (due to incomplete surgical resection)
  • Tumors that recur after surgery 
  • Tumors that have spread to distant parts of the body 

How can Malignant Adenomyoepithelioma of Breast be Prevented?

Currently, no specific preventive measures are available for Malignant Adenomyoepithelioma of Breast. In general, it is important to be aware of the following:

• Learning to do regular ‘breast self-exams’ in order to help identify any unusual lumps or signs in the breast
• Maintain a healthy body weight and exercise regularly
• Implement and follow a well-balanced diet; a high intake of fiber via fresh fruits and vegetables helps in a healthy lifestyle
• Avoid or completely stop smoking
• If taking alcohol, only drink in moderation; limit to one or (maximum) two drinks a day
• Limit combination hormone therapy used to treat symptoms of menopause. It is advised that individuals be aware of the potential benefits and risks of such hormonal medications
• Cancer screenings can help detect any breast cancer during the early stages

What is the Prognosis of Malignant Adenomyoepithelioma of Breast? (Outcomes/Resolutions)

• Malignant Adenomyoepithelioma of Breast is a very uncommon type of breast cancer. The prognosis of the condition generally depends upon the stage of the tumor
• It is estimated that one-third of the patients with Mammary Malignant Adenomyoepithelioma have distant metastasis or local recurrences. This usually occurs anywhere from 4 months to 3 years following the first diagnosis
• The prognosis of breast cancer, in general, depends upon a set of several factors that include: 
  • The grade of the breast tumor such as grade1, grade2, and grade 3. Grade1 indicates a well-defined tumor, whereas grade 3 indicates a poorly-defined tumor
  • The size of the breast tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
  • Stage of breast cancer: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
  • Cell growth rate
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Individuals with bulky disease of the breast cancer have a poorer prognosis
  • Involvement of the lymph node, which can adversely affect the prognosis
  • Involvement of vital organs may complicate the condition
  • The surgical resectability of the tumor (meaning if the tumor can be removed completely)
  • Whether the tumor is occurring for the first time or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur 
  • Response to treatment of breast cancer: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
  • Progression of the condition makes the outcome worse
• An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment
• The combination chemotherapy drugs used, may have some severe side effects (like cardio-toxicity). These chiefly impacts the elderly adults, or those who are already affected by other medical conditions. Tolerance to the chemotherapy sessions is a positive influencing factor

Additional and Relevant Useful Information for Malignant Adenomyoepithelioma of Breast:

The following DoveMed website links are useful resources for additional information:

https://www.dovemed.com/healthy-living/womens-health/

https://www.dovemed.com/diseases-conditions/cancer/