Rare Dermatology News

Disease Profile

Phyllodes tumor of the breast

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Adult

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ICD-10

D48.6

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Phyllodes breast tumor; Cystosarcoma phyllodes of the breast; Cystosarcoma phyllodes;

Categories

Rare Cancers

Summary

Phyllodes tumors of the breast are rare tumors that start in the connective (stromal) tissue of the breast.[1][2] They get their name from the leaf-like pattern in which they grow (phyllodes means leaf-like in Greek).[2][3] They are most common in women in their 30s and 40s, although women of any age can be affected. These tumors, which are usually painless, tend to grow quickly, but rarely spread outside of the breast.[1][2] Most phyllodes tumors are benign. About 1 in 10 are cancerous.[1] The underlying cause of these tumors in unknown.[3] Surgery is the main treatment. Because the tumors can reoccur if they are not removed with enough surrounding tissue, the tumor and at least 1 cm of tissue should be removed. Cancerous phyllodes tumors are often treated with mastectomy. Close follow-up with frequent breast examinations are recommended after surgery.[1][2]

Treatment

Surgery is the main treatment for phyllodes tumors of the breast. This is the case regardless of whether they are benign or malignant. Because these tumors can come back if enough normal tissue is not removed, surgery should involve removing the tumor and at least 1 cm of the surrounding tissue.[1][2] Some doctors feel that an even wider margin of healthy tissue should be removed (wide excision).[2][3] Malignant phyllodes tumors may be treated more aggressively, with removal of wider margins of tissue or removal of part or all of the breast (partial or total mastectomy).[1][2] Because spread to the underarm lymph nodes is rare, it is usually not necessary to remove them.[2]

Phyllodes tumors of the breast do not respond to hormone therapy.[1][2][3] Radiation and chemotherapy are not typically used as there is little evidence that these methods are effective for phyllodes tumors.[2][3]

Because phyllodes tumors can come back, close follow-up with frequent breast examinations and imaging are recommended following surgery.[1][2][3] This may include self and clinical breast exams, mammograms, ultrasound of the breast, MRI of the breast, and/or CT scans of the chest and abdomen (especially in malignant or metastatic cases).[2]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Social Networking Websites

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • The American Cancer Society provides information on Phyllodes tumor of the breast. Please click on the link to access this resource.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
        • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Phyllodes tumor of the breast. Click on the link to view a sample search on this topic.

          References

          1. Phyllodes tumors. American Cancer Society. April 21, 2016; https://www.cancer.org/healthy/findcancerearly/womenshealth/non-cancerousbreastconditions/non-cancerous-breast-conditions-phyllodes-tumors.
          2. Phyllodes Tumors of the Breast. BreastCancer.org. April 6, 2016; https://www.breastcancer.org/symptoms/types/phyllodes.
          3. Lannin DR. Phyllodes Tumor (Cystosarcoma Phyllodes). Medscape Reference. June 20, 2016; https://emedicine.medscape.com/article/188728-overview.
          4. Grau AM, Chakravarthy AB, Chugh R. Phyllodes tumors of the breast. UpToDate. February 20, 2014; https://www.uptodate.com/contents/phyllodes-tumors-of-the-breast.

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