Phyllodes tumor of the breast

Phyllodes tumor
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Phyllodes tumor of the breast - a benign, malignant, or borderline circumscribed biphasic neoplasm that arises from the breast. It usually affects middle aged women. It is characterized by the presence of a double layer of epithelial cells that are arranged in clefts, surrounded by a spindle cell mesenchymal (stromal) component. It is often large and bulky and grows quickly. It may be benign (not cancer) or malignant (cancer) and may spread to other parts of the body. Also called CSP and cystosarcoma phyllodes.

Alternate names

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

Etiology

They get their name from the leaf-like pattern in which they grow (phyllodes means leaf-like in Greek).

Clinical features

Phyllodes tumors of the breast are rare tumors that start in the connective (stromal) tissue of the breast. 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.

Most phyllodes tumors are benign. About 1 in 10 are cancerous.

Cause

The underlying cause of these tumors in unknown. Surgery is the main treatment.

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. Cancerous phyllodes tumors are often treated with mastectomy. Close follow-up with frequent breast examinations are recommended after surgery.

Some doctors feel that an even wider margin of healthy tissue should be removed (wide excision). 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). Because spread to the underarm lymph nodes is rare, it is usually not necessary to remove them.

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

Follow up

Because phyllodes tumors can come back, close follow-up with frequent breast examinations and imaging are recommended following surgery. 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).

Prognosis

Phyllodes tumors that are benign have an excellent prognosis following surgery. However, local recurrence is possible. If the tumor recurs locally, further surgery, including local excision or partial or total mastectomy typically results in a good outcome.

3 and 5 year survival

The SArcoma and PHYllode Retrospective (SAPHYR) Study reported a three year survival rate for benign and borderline phyllodes tumors of 100 percent. Other studies have reported similar findings. Malignant tumors have a higher chance of coming back. If the tumor metastasizes, common locations include the lung, mediastinum, and skeleton. The five year survival rate for malignant phyllodes tumors has been reported to be 60 to 80 percent.

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Classification	ICD-10: C50 , D24 , D48.6 MeSH: D003557 DiseasesDB: 3396
External resources	eMedicine: med/500