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

• Haemangiopericytoma of Breast
• HPC of Breast
• Mammary Hemangiopericytoma

What is Hemangiopericytoma 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
• Hemangiopericytoma (HPC) of Breast is a rare, low-grade malignant tumor of the mesenchyme. Most cases have been observed in adult women, though some cases have been reported of young children and even men
• Generally, a malignancy in mesenchymal cells leads to the formation of a sarcoma, and hence, hemangiopericytoma can be termed as a type of soft tissue sarcoma. The mesenchyme are cells that form various components of the body including the connective tissues, lymphatic and circulatory system tissues
• The signs and symptoms of Hemangiopericytoma of Breast include lump in the breast, swelling or skin thickening around the region of the lump, and change in breast profile. Complications from this cancer type may include treatment side effects causing nausea, vomiting, and hair loss
• In order to treat Hemangiopericytoma 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
• The prognosis of Hemangiopericytoma of Breast is good with suitable treatment, since a majority of these tumors behave in a benign manner. However, tumors over 5 cm in size may be more aggressive

Who gets Hemangiopericytoma of Breast? (Age and Sex Distribution)

• Hemangiopericytoma of Breast is a very rare breast cancer tumor; less than 50 cases have been reported worldwide
• It is seen in young, middle-aged, and older women, in the 22-67 year age range. However, rarely men and young children (5-7 years) are also known to be affected
• 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 Hemangiopericytoma of Breast? (Predisposing Factors)

• No clear-cut risk factors for Hemangiopericytoma of Breast have been established to date
• However, women have a higher risk for developing the condition than men and children

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 Hemangiopericytoma of Breast? (Etiology)

• The exact cause of development of Hemangiopericytoma 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 Hemangiopericytoma of Breast?

The signs and symptoms of Hemangiopericytoma of Breast may include:

• A lump in the breast or underarm area that is normally detected on a screening mammogram; skin involvement is not seen
• The size of the tumor may range from 1 cm to 19 cm; thus, the tumor can grow to large sizes
• The tumors are solid, spherical or oval, and well-defined
• There may not be any pain associated with the tumor
• 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 

How is Hemangiopericytoma of Breast Diagnosed?

Hemangiopericytoma 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 Mammary Hemangiopericytoma. 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

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 Hemangiopericytoma of Breast?

The complications of Hemangiopericytoma of Breast may include:

• Emotional distress due to the presence of breast cancer
• Metastasis of the tumor to local and regional sites; spread of tumor to lymph node is rare
• Recurrence of the tumor (locally)
• 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 Hemangiopericytoma of Breast Treated?

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

• Type of cancer
• The staging of the cancer: If breast cancer is diagnosed, staging helps determine whether it has spread and which treatment options are best for the patient
• Whether the cancer cells are sensitive to certain particular hormones, and
• Personal preferences

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 Hemangiopericytoma 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 Hemangiopericytoma of Breast)
  • Tumors that have spread to distant parts of the body (metastatic Hemangiopericytoma of Breast)

How can Hemangiopericytoma of Breast be Prevented?

The development of Hemangiopericytoma of Breast is difficult to prevent. Currently, no specific preventive measures are available to avoid Mammary Hemangiopericytoma.

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 Hemangiopericytoma of Breast? (Outcomes/Resolutions)

• Hemangiopericytoma of Breast is a malignant type of breast cancer. However, since many tumors behave benignly, the prognosis of the condition is generally good. The same may not hold true when tumors are larger than 5 cm in size
• Among the cases observed, no recurrences or metastasis has been noted. Healthcare providers recommend a complete surgical excision of the tumor as the preferred treatment option
• 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 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
• 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 Hemangiopericytoma of Breast:

• Japan is an exception of a developed nation with lowered cases of breast cancer, unlike European nations and America

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/