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Leiomyosarcoma (LMS) of Breast is a rare type of malignant connective tissue tumor. Leiomyosarcoma occurs in the muscles that are not voluntarily controlled, known as smooth muscles.

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

  • LMS of Breast
  • Mammary Leiomyosarcoma
  • Mammary LMS

What is Leiomyosarcoma 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
  • Leiomyosarcoma (LMS) of Breast is a rare type of malignant connective tissue tumor. Leiomyosarcoma occurs in the muscles that are not voluntarily controlled, known as smooth muscles. Due to the bounty of smooth muscle throughout the body, any individual is susceptible to leiomyosarcoma
  • The signs and symptoms of Leiomyosarcoma of Breast include an enlarging mass in the breast, pain around the region of the lump, and change in breast profile due to large-sized tumors
  • 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 Leiomyosarcoma 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 Leiomyosarcoma of Breast is guarded despite treatment, since it is an invasive type of malignancy. However, early diagnosis and adequate treatment can significantly improve the outcomes

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

  • Leiomyosarcoma of Breast constitutes much less than 1% of all breast tumor types
  • The age of presentation is usually between 30-70 years; though it can be infrequently seen in older children, teens, and much older adults (over 80 years of age)
  • Both males and females are affected, though a female predominance is observed
  • All racial and ethnic groups are affected and no specific predilection is seen
  • Developed countries (the affluent nations) show a higher prevalence rate 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 Leiomyosarcoma of Breast? (Predisposing Factors)

  • No clear-cut risk factors for Leiomyosarcoma 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 Leiomyosarcoma of Breast? (Etiology)

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

The signs and symptoms of Leiomyosarcoma of Breast may include:

  • A benign slow-growing lump in the breast; typically, only one breast is affected
  • The tumors are firm, well-defined (with infiltrating margins) and can be felt by touch
  • The tumors may ‘move around’ and may be painful
  • Small tumors remain asymptomatic and may be missed during a mammogram screening
  • The tumor size may range from a few mm to nearly 15 cm, though most leiomyosarcomas may be bigger in size during diagnosis
  • 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 Leiomyosarcoma of Breast Diagnosed?

Leiomyosarcoma 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 Leiomyosarcoma. 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 Leiomyosarcoma of Breast?

The complications of Leiomyosarcoma of Breast may include:

  • Emotional distress due to the presence of breast cancer
  • Metastasis of the tumor to local and regional sites
  • Recurrence of the tumor may take place after a long duration following excisional surgery
  • 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 Leiomyosarcoma of Breast Treated?

Treatment options available for individuals with Leiomyosarcoma 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 (with wide margins) is the most common form of treatment involving the removal of the tumor and is the treatment of choice. 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 Leiomyosarcoma 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 Leiomyosarcoma of Breast)
    • Tumors that have spread to distant parts of the body (metastatic Leiomyosarcoma of Breast)

How can Leiomyosarcoma of Breast be Prevented?

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

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 Leiomyosarcoma of Breast? (Outcomes/Resolutions)

  • Leiomyosarcoma of Breast is a type of invasive breast cancer. The prognosis of the condition is generally guarded, since the tumors are aggressive
  • 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 for Leiomyosarcoma 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/

What are some Useful Resources for Additional Information?

Breast Cancer Education Association
1027 W. Roselawn Avenue, Roseville, MN 55113
Phone: (651) 487-3578
Email: info@breastcancereducation.org
Website: http://breastcancereducation.org

American Cancer Society (ACS)
1599 Clifton Road, NE Atlanta, GA 30329-4251
Toll-Free: (800) 227-2345
TTY: (866) 228-4327
Website: http://www.cancer.org

National Cancer Institute (NCI)
U.S. National Institutes of Health
Public Inquiries Office
Building 31, Room 10A03
31 Center Drive, MSC 8322 Bethesda, MD 20892-2580
Phone: (301) 435-3848
Toll-Free: (800) 422-6237
TTY: (800) 332-8615
Email: cancergovstaff@mail.nih.gov
Website: http://www.cancer.gov

References and Information Sources used for the Article:

Lakhani, S. R. (Ed.). (2012). WHO classification of tumours of the breast. International Agency for Research on Cancer.

http://womenshealth.gov/breast-cancer/what-is-breast-cancer/index.html (accessed on 06/20/16)

http://www.cdc.gov/cancer/breast/basic_info/index.htm (accessed on 06/20/16)

http://www.mayoclinic.com/health/breast-cancer/DS00328 (accessed on 06/20/16)

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3318164/ (accessed on 06/20/16)

http://breast-cancer.ca/leiomy/ (accessed on 06/20/16)

Helpful Peer-Reviewed Medical Articles:

Henry, E., Villalobos, V., Million, L., Jensen, K. C., West, R., Ganjoo, K., ... & Telli, M. L. (2012). Chest wall leiomyosarcoma after breast-conservative therapy for early-stage breast cancer in a young woman with Li-Fraumeni syndrome. Journal of the National Comprehensive Cancer Network, 10(8), 939-942.

Rane, S. U., Batra, C., & Saikia, U. N. (2012). Primary leiomyosarcoma of breast in an adolescent girl: a case report and review of the literature.Case reports in pathology, 2012.

Olcina, M., Merck, B., Giménez-Climent, M. J., Almenar, S., Sancho-Merle, M. F., Llopis, F., & Vázquez-Albadalejo, C. (2008). Radiation-induced leiomyosarcoma after breast cancer treatment and TRAM flap reconstruction. Sarcoma, 2008.

Kamio, T., Nishizawa, M., Aoyama, K., Ohchi, T., Nishikawa, T., Kobayashi, M., & Kameoka, S. (2010). Primary leiomyosarcoma of the breast treated by partial resection of the breast including nipple and areola: report of a case. Surgery today, 40(11), 1063-1067.

Székely, E., Madaras, L., Kulka, J., Járay, B., & Nagy, L. (2001). Leiomyosarcoma of the female breast. Pathology Oncology Research,7(2), 151-153.

Munitiz, V., Rios, A., Canovas, J., Ferri, B., Sola, J., Canovas, P., ... & Parrilla, P. (2004). Primitive leiomyosarcoma of the breast: case report and review of the literature. The breast, 13(1), 72-76.

Gupta, R. K., Kenwright, D., Naran, S., Lallu, S., & Fauck, R. (2000). Fine needle aspiration cytodiagnosis of leiomyosarcoma of the breast. Acta cytologica, 44(6), 1101-1105.

Lin, C. H., Yeh, C. N., & Chen, M. F. (2003). Breast metastasis from uterine leiomyosarcoma: a case report. Archives of gynecology and obstetrics,267(4), 233-235.