Malignant Phyllodes Tumor of Breast

Malignant Phyllodes Tumor of Breast

Article
Women's Health
Diseases & Conditions
+1
Contributed byMaulik P. Purohit MD MPHNov 05, 2018

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

  • Malignant Mammary Phyllodes Tumor
  • Malignant Phyllodes Tumour of Breast
  • Malignant Phylloides Tumor of Breast

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

  • Malignant Phyllodes Tumor of Breast is a rare tumor of the breast, which is observed in middle-aged individuals (mostly women). The most common site of phyllodes tumor is the breast
  • In general, phyllodes tumor may be classified as benign, malignant, or borderline tumor based on their behavior. Some tumors are malignant and behave aggressively; they may infiltrate into surrounding tissue structures and metastasize to other body sites
  • The cause of Malignant Phyllodes Tumor of Breast is unknown, but the general risk factors may include gender (women are affected more than men), the use of birth control pills, obesity, and one’s reproductive history
  • The signs and symptoms of Malignant Phyllodes Tumor of Breast may include the presence of a rapidly-growing solitary tumor in the breast. The complications are dependent upon the stage of the cancer and may also be associated with the treatment employed
  • Malignant Phyllodes Tumor of Breast is treated through a surgical excision, per the healthcare provider’s recommendation. The prognosis depends on the cancer stage, and overall health of the individual. In general, the prognosis of the tumor is excellent with early detection and prompt treatment

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

  • Malignant Phyllodes Tumor of Breast are rare tumors affecting individuals over a wide age range
  • Generally, it affects women between the age of 40 and 50 years (prior to or around menopause). However, malignant phyllodes tumors are seen to affect slightly older women
  • All racial and ethnic groups are affected and no specific predilection is seen
  • Studies inform that much less than 1% of all primary breast tumors constitute Phyllodes Tumor of Breast

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

The definitive risk factors for Malignant Phyllodes Tumor of Breast are not well-understood. However, the following risk factors increase one’s chances of getting benign breast tumors, and these include:

  • Gender: Women have a higher risk for developing the condition than men
  • Obesity: Being overweight or obese increases the risk
  • Alcohol consumption
  • Menstrual cycle: Women who got their period before the age of 12 years, and those who reached menopause after age 55 have a higher risk. The longer the duration between menarche and menopause, the greater is the risk. This is due to hormonal influences during the reproductive period on the breast tissue
  • Postmenopausal hormone therapy: Women taking hormone replacement therapy medications containing both estrogen and progesterone for menopause, have a higher risk
  • Reproductive history: Having the first child after the age of 35, or never having a child
  • Birth control pills
  • Physical inactivity: A lack of physical exercise (leading a sedentary life) can increase one’s risk 
  • Not breastfeeding the child

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones 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 Phyllodes Tumor of Breast? (Etiology)

  • The exact cause of development of Malignant Phyllodes Tumor of Breast is currently not clearly understood
  • Studies have shown that such tumors may be caused by hormonal influence

What are the Signs and Symptoms of Malignant Phyllodes Tumor of Breast?

The signs and symptoms of Malignant Phyllodes Tumor of Breast may include:

  • The presence of a rapidly growing lump in the breast; the tumors may develop over few weeks to months
  • Most tumors are solitary, ill-defined, and range in size between 2-3 cm
  • Generally, the tumors are painless to touch
  • If left untreated, the rapidly-growing tumors may cause breast skin ulceration resulting in an open wound
  • Ulceration of skin can lead to pain and discomfort
  • Changes to the skin covering the breast or nipple area, including dimpling, irritation, redness, scaling, peeling, or puckering
  • The presence of multiple tumors are rarely observed

It is important to note that both benign and malignant phyllodes tumors are known to grow rapidly.

How is Malignant Phyllodes Tumor of Breast Diagnosed?

Malignant Phyllodes Tumor of Breast may be diagnosed in the following manner:

  • Complete physical examination with comprehensive medical and family history evaluation
  • The following information may be sought by the healthcare provider:
    • Family history of breast cancer and ovarian cancer
    • Family history of BRCA 1 or BRCA 2 mutation
    • History of pregnancy
    • History of breastfeeding (lactational history)
    • Prior history of breast cancer (if any)
    • Prior history of breast trauma
    • Prior history of breast biopsy/surgery; history of any breast implantation may also be included
    • History of radiation therapy (in the past)
    • History of chemotherapy (in the past)
    • History of hormonal therapy (in the past)
    • History of autoimmune disorders
  • Breast exam to check for any lumps or unusual signs in the breasts
  • Blood tests including complete blood count (CBC)
  • Mammogram: A mammogram uses X-rays to provide images of the breast. These benign tumors are identified as a mammogram mass, which may or may not be associated with microcalcification. The mammography findings may raise enough suspicion to warrant a tissue biopsy
  • Breast ultrasound scan: Using high-frequency sound waves to produce images of the breast, the type of tumor, whether fluid-filled cyst or solid mass type, may be identified
  • 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. FNAB cannot help definitively diagnose Phyllodes Tumor of Breast. It only helps determine if the tumor is malignant or benign. This can help the healthcare provider discuss and plan the next steps (with respect to diagnosis and treatment)
  • 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

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 Phyllodes Tumor of Breast?

The complications of Malignant Phyllodes Tumor of Breast may include:

  • Emotional distress due to the presence of breast cancer
  • Ulceration of overlying skin may lead to secondary bacterial and/or fungal infections
  • Metastasis of the tumor to local and regional sites including to lymph nodes and skin
  • Recurrence of the tumor on incomplete surgical removal
  • 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 Phyllodes Tumor of Breast Treated?

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

  • Type of cancer
  • The staging of the cancer
  • Whether the cancer cells are sensitive to certain particular hormones, and
  • Personal preferences

In general, breast cancer stages range from 0 to IV. 0 may indicate a small and non-invasive cancer, while IV indicates that the cancer has spread to other areas of the body. Briefly, as per National Cancer Institute (at the National Institutes of Health), breast cancer is staged as follows:

  • Stage 0 (carcinoma in situ): The abnormal cancer cells are confined to their site of origin (the breast duct linings, lobules, or nipples)
  • Stage I: The tumor is 2 centimeters in diameter or less, and has not spread outside the breast
  • Stage II: The tumor may be up to 5 centimeters in diameter and may have spread to lymph nodes. Another criteria is that the tumor may be larger than 5 centimeters in diameter, but has not spread to surrounding lymph nodes
  • Stage III: The tumor may be more than 5 centimeters in diameter and may have spread to several axillary lymph nodes, or to the lymph nodes near the breastbone. The cancer may also have spread to the breast skin/chest wall, causing ulcer-like sores, or a swelling
  • Stage IV: The tumor has spread outside the breast and to other organs, such as the bones, liver, lungs, or brain, regardless of its size

If breast cancer is diagnosed, staging helps determine whether it has spread and which treatment options are best for the patient. In case of a malignancy, a combination of surgery, chemotherapy, and radiation therapy may be necessary.

The treatment methods for Malignant Phyllodes Tumor of Breast are described below:

Surgery: Surgery is the most common form of treatment involving the removal of the tumor. Various types of surgery, to remove the cancer 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

Chemotherapy: Using the help of drugs to either kills the cancer cells or shrink the tumor.

  • Chemotherapy may be recommended after surgery, for a tumor with a high probability of returning or metastasizing (spreading) in what is termed as adjuvant chemotherapy. The procedure kills any remaining cancer cells, following a surgery for the cancer
  • Chemotherapy may also be suggested before surgery, for women with large cancers to reduce the size of the tumor. This allows for easier removal during surgery and is termed neoadjuvant chemotherapy
  • The use of chemotherapy is also an option for women with advanced cancer, which that has already spread outside the breast

Radiation therapy: Using high-energy beams, including x-rays, radiation therapy aims to kill cancerous cells.

  • Radiation therapy can be done externally (external beam radiation) using a large machine, to target rays at the cancerous area
  • It may also be administered internally (internal beam radiation) using a radioactive substance that collects at the site, where the cancer originated
  • It is commonly recommended after a lumpectomy, when the cancer is still in its early stages

Follow-up care with frequent breast self-examinations and screening mammograms may be recommended by the healthcare provider.

How can Malignant Phyllodes Tumor of Breast be Prevented?

Malignant Phyllodes Tumor of Breast is difficult to prevent. Since prevention is generally not possible, it is important to be aware of the risk factors for breast lumps and take steps to avoid them.

  • The individual must regularly conduct breast self-exams, to ensure that no lumps are present
  • 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
  • Drink alcohol 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 hormone therapy

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

  • The prognosis of Malignant Phyllodes Tumor of Breast depends upon a set of several factors that include:
    • 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 respectability 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
  • In general, the prognosis of Malignant Mammary Phyllodes Tumor is excellent with early adequate treatment (complete excision and tumor removal); although, the tumor is highly-susceptible to recur
  • The combination chemotherapy drugs used, may have some severe side effects (like cardio-toxicity). This 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 Phyllodes Tumor of Breast:

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

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

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

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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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