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

• Primary Mammary Carcinoma, Squamous Cell Type
• Primary Mammary Squamous Cell Carcinoma
• Primary Squamous Cell Breast Carcinoma

What is Primary Squamous Cell Carcinoma 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 which include ductal carcinoma and lobular carcinoma
• Primary Squamous Cell Carcinoma of Breast is a very rare, invasive, primary breast tumor that presents squamous pattern. It belongs to a rare group of tumors known as metaplastic carcinomas of breast. These types are mostly observed in middle-aged and elderly women
• The signs and symptoms of Primary Squamous Cell Carcinoma of Breast include the presence of a lump in the breast, swelling or skin thickening around the region of the lump, and change in breast profile
• Complications from this cancer type includes the spread of cancer from the breast to other locations and treatment side effects that may include nausea, vomiting, and hair loss
• In order to treat Primary Squamous Cell Carcinoma of Breast, the healthcare provider may use a combination of therapies that may include surgery, chemotherapy, and radiation therapy, depending on the stage of the tumor. However, the tumor does not respond well to treatment
• The prognosis of Primary Squamous Cell Carcinoma of Breast is poor, since it is an aggressive type of malignancy. However, early diagnosis and adequate treatment can significantly improve the outcomes

Who gets Primary Squamous Cell Carcinoma of Breast? (Age and Sex Distribution)

• Primary Squamous Cell Carcinoma of Breast represents less than 0.5-1% of all breast cancer types and is a very rare condition
• The mean age of presentation in women is around 55 years, but a wide age range (30-80 years) is seen
• All racial and ethnic groups are affected and no specific predilection is seen
• Developed countries (the affluent nations) show a higher prevalence rate for breast cancer than developing countries. Thus, America, Europe, Australia have greater incidences than Asia (including India, China, and Japan) and Africa

What are the Risk Factors for Primary Squamous Cell Carcinoma of Breast? (Predisposing Factors)

• No clear-cut risk factors for Primary Squamous Cell Carcinoma of Breast have been established to date

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 Primary Squamous Cell Carcinoma of Breast? (Etiology)

• The exact cause of development of Primary Squamous Cell Carcinoma of Breast is currently not clearly known
• Certain gene mutations have also been reported in the tumors. Research is being performed to determine how these mutations contribute to the formation of the tumors

What are the Signs and Symptoms of Primary Squamous Cell Carcinoma of Breast?

The signs and symptoms of Primary Squamous Cell Carcinoma of Breast may include:

• A lump in the breast or underarm area
• The tumor is a well-defined and firm/solid mass
• The average size is 3-5 cm; it can grow to large sizes
• 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)
• Bloody discharge from the nipple
• Changes to the skin covering the breast or nipple area, including dimpling, irritation, redness, scaling, peeling, or puckering
• In some cases, pain in the breast

How is Primary Squamous Cell Carcinoma of Breast Diagnosed?

Primary Squamous Cell Carcinoma 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)
• 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
• 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, 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 Primary Squamous Cell Carcinoma 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. A definitive diagnosis on a core biopsy may be difficult. Hence, a follow-up surgical procedure to obtain a larger breast biopsy specimen (such as through a lumpectomy) is often performed
• 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

Based on a histopathological examination of the breast biopsy specimen, a pathologist can determine the subtype of squamous cell carcinoma the tumor. Depending on the pattern noted by the pathologist, there are 3 variants:

• Large cell keratinizing variant
• Spindle cell variant
• Acantholytic variant

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 Primary Squamous Cell Carcinoma of Breast?

The complications of Primary Squamous Cell Carcinoma of Breast may include:

• Emotional distress due to the presence of breast cancer
• Metastasis of the tumor to local and regional sites
• 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 Primary Squamous Cell Carcinoma of Breast Treated?

Treatment options available for individuals with Primary Squamous Cell Carcinoma 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.

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

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 (recurrent Squamous Cell Carcinoma of Breast) 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 (recurrent Squamous Cell Carcinoma of Breast)
  • Tumors that have spread to distant parts of the body (metastatic Squamous Cell Carcinoma of Breast)

How can Primary Squamous Cell Carcinoma of Breast be Prevented?

The development of Primary Squamous Cell Carcinoma of Breast is difficult to prevent. Currently, no specific preventive measures are available to avoid Primary SCC of Breast.

In general, however, it is important to be aware of certain risk factors for breast tumors, which include:

• Maintain a healthy weight and exercise regularly; physical activity can reduce risk, especially in post-menopausal women
• Implement and follow a well-balanced diet; a high intake of fiber via fresh fruits and vegetables can reduce the risk
• 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
• Cancer screenings can help detect any breast cancer, at its earliest stages
• Learn to do ‘breast self-exams’, in order to help identify any unusual lumps, signs in the breasts

What is the Prognosis of Primary Squamous Cell Carcinoma of Breast? (Outcomes/Resolutions)

• Primary Squamous Cell Carcinoma of Breast is a type of invasive breast cancer. The prognosis of the condition is generally guarded, since the tumors are usually aggressive
• The acantholytic form of SCC is more aggressive than the other large cell keratinizing and spindle cell variant, and hence, may have a poorer prognosis
• 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). 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 Primary Squamous Cell Carcinoma of Breast:

• Japan is an exception of a developed nation with lowered cases of breast cancer, unlike European nations and America
• Current studies have shown that aromatase inhibitors, medications that block estrogen hormonal effects in the body, reduce the risk of recurrence of breast cancer. Recent studies (ASCO 2016, Chicago) have shown that treatment using aromatase inhibitors can be given up to 10 years without affecting the quality of life of women
• Tumors that are negative for estrogen receptor, progesterone receptor, and HER2/neu have worse prognosis. Such tumors are called “triple-negative” tumors

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/