Phyllodes Tumor
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Phyllodes tumors (from
Greek Greek may refer to: Greece Anything of, from, or related to Greece, a country in Southern Europe: *Greeks, an ethnic group. *Greek language, a branch of the Indo-European language family. **Proto-Greek language, the assumed last common ancestor ...
: ''phullon'' leaf), also cystosarcoma phyllodes, cystosarcoma phylloides and phylloides tumor, are typically large, fast-growing masses that form from the periductal
stromal cell Stromal cells, or mesenchymal stromal cells, are differentiating cells found in abundance within bone marrow but can also be seen all around the body. Stromal cells can become connective tissue cells of any organ, for example in the uterine mucosa ...
s 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 A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
.


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 Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malign ...
, borderline, or
malignant Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...
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 The International Classification of Diseases for Oncology (ICD-O) is a domain-specific extension of the ICD, International Statistical Classification of Diseases and Related Health Problems for tumor diseases. This classification is widely used by ...
, but not by
MeSH A mesh is a barrier made of connected strands of metal, fiber, or other flexible or ductile materials. A mesh is similar to a web or a net in that it has many attached or woven strands. Types * A plastic mesh may be extruded, oriented, ex ...
. Younger women have a higher chance of having a benign phyllodes tumor.


Treatment

The common treatment for phyllodes is
wide local excision A wide local excision (WLE) is a surgical procedure to remove a small area of diseased or problematic tissue with a margin of normal tissue. This procedure is commonly performed on the breast and to skin lesions, but can be used on any area of the ...
. 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 Breast-conserving surgery (BCS) refers to an operation that aims to remove breast cancer while avoiding a mastectomy. Different forms of this operation include: lumpectomy (tylectomy), wide local excision, segmental resection, and quadrantectom ...
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