Phyllodes Tumors
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Phyllodes tumors (from Greek: ''phullon'' leaf), also cystosarcoma phyllodes, cystosarcoma phylloides and phylloides tumor, are typically large, fast-growing masses that form from the periductal stromal cells of the
breast The breast is one of two prominences located on the upper ventral region of a primate's torso. Both females and males develop breasts from the same embryological tissues. In females, it serves as the mammary gland, which produces and secret ...
. They account for less than 1% of all breast neoplasms.


Presentation

This is predominantly a tumor of adult women, with very few examples reported in adolescents. Patients typically present with a firm, palpable mass. These tumors are very fast-growing, and can increase in size in just a few weeks. Occurrence is most common between the ages of 40 and 50, prior to
menopause Menopause, also known as the climacteric, is the time in women's lives when menstrual periods stop permanently, and they are no longer able to bear children. Menopause usually occurs between the age of 47 and 54. Medical professionals often d ...
. This is about 15 years older than the typical age of patients with
fibroadenoma Fibroadenomas are benign breast tumours characterized by an admixture of stromal and epithelial tissue. Breasts are made of lobules (milk producing glands) and ducts (tubes that carry the milk to the nipple). These are surrounded by glandular, ...
, a condition with which phyllodes tumors may be confused. They have been documented to occur at any age below 12 years.


Diagnosis


Classification

Phyllodes tumors are a
fibroepithelial tumor A fibroepithelial neoplasm (or tumor) is a biphasic tumor. They consist of epithelial tissue, and stromal or mesenchymal tissue. They may be benign or malignant.Tavassoli, F.A., Devilee, P. (Eds). 2003. World Health Organization Classification o ...
composed of an epithelial and a cellular stromal component. They may be considered benign, borderline, or malignant depending on histologic features including stromal cellularity, infiltration at the tumor's edge, and mitotic activity. All forms of phyllodes tumors are regarded as having malignant potential. A large series from the M.D. Anderson Cancer Centre reported the incidence of each as benign (58%), borderline (12%), and malignant (30%). Malignant phyllodes tumours behave like sarcomas and can develop blood-borne metastases. Approximately 10% of patients with phyllodes tumours develop distant metastases and this can go up to 20% in patients with histologically malignant tumours. The commonest sites for distant metastases are the lung, bone, and abdominal viscera. Rare sites of metastasis, such as to the parotid region, have also been described. They are classified as a
fibroepithelial tumor A fibroepithelial neoplasm (or tumor) is a biphasic tumor. They consist of epithelial tissue, and stromal or mesenchymal tissue. They may be benign or malignant.Tavassoli, F.A., Devilee, P. (Eds). 2003. World Health Organization Classification o ...
by ICD-O, but not by MeSH. Younger women have a higher chance of having a benign phyllodes tumor.


Treatment

The common treatment for phyllodes is wide local excision. Other than surgery, there is no cure for phyllodes, as chemotherapy and radiation therapy are not effective. The risk of developing local recurrence or
metastases Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then, ...
is related to the histologic grade, according to the above-named features. Despite wide excision, a very high percentage of surgeries yielded incomplete excision margins that required revision surgery. Radiation treatment after breast-conserving surgery with negative margins may significantly reduce the local recurrence rate for borderline and malignant tumors. The authors of a 2012 study have derived a risk calculator for relapse risk of phyllodes tumors after surgery.This is available at www.phyllodes.com.


Spectrum

Phyllodes tumors are considered to be on a
spectrum A spectrum (plural ''spectra'' or ''spectrums'') is a condition that is not limited to a specific set of values but can vary, without gaps, across a continuum. The word was first used scientifically in optics to describe the rainbow of colors i ...
of disease that consists of
fibroadenoma Fibroadenomas are benign breast tumours characterized by an admixture of stromal and epithelial tissue. Breasts are made of lobules (milk producing glands) and ducts (tubes that carry the milk to the nipple). These are surrounded by glandular, ...
, fibroadenoma variant and benign phyllodes. Some would extend the spectrum to include malignant phyllodes tumors and frank
sarcoma A sarcoma is a malignant tumor, a type of cancer that arises from transformed cells of mesenchymal (connective tissue) origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or hematopoietic tissues, and sarcom ...
.


References


External links

{{Breast neoplasia Breast neoplasia